Peripheral T-Cell Lymphoma

MONDO:0015760 Pathograph 13 Show in embeddings browser T-cell lymphoma

Peripheral T-cell lymphoma (PTCL) is a clinically used family of aggressive mature T-cell non-Hodgkin lymphomas dominated in nodal and systemic practice by PTCL-not otherwise specified (PTCL-NOS), nodal T-follicular helper (TFH) lymphomas, and systemic anaplastic large cell lymphomas (ALCL). PTCL biology is heterogeneous, with subtype-weighted contributions from TFH-lineage epigenetic lesions, RHOA/VAV1-mediated T-cell receptor signaling, PTCL-NOS GATA3/TBX21 transcriptional programs, and an immune microenvironment enriched in regulatory and exhausted T cells. Following the cancer-modeling guidance from dismech issue # 1198, this entry treats PTCL as a single mechanism-graph unit and keeps clinically meaningful histologic entities as flat subtype facets rather than separate dismech pages. Because MONDO lacks an exact family-level peripheral T-cell lymphoma umbrella term, the page is anchored to the closest MONDO family class and refined with subtype-specific NCIT histopathology and oncology treatment terms.

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1
Mappings
1
Definitions
8
Pathophys.
7
Histopath.
6
Phenotypes
13
Pathograph
7
Genes
6
Medical Actions
6
Subtypes
2
Datasets
1
Deep Research
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Classifications

ICD-O Morphology
Lymphoma
Harrison's Part
ONCOLOGY HEMATOLOGY
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Mappings

MONDO
MONDO:0015760 T-cell non-Hodgkin lymphoma
skos:closeMatch MONDO
MONDO does not currently provide an exact family-level peripheral T-cell lymphoma umbrella term. This entry therefore anchors to the closest MONDO T-cell non-Hodgkin lymphoma family class, while the PTCL-specific histology axis is carried by flat subtype facets and NCIT-grounded histopathology.
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Definitions

1
Family-level clinical definition of peripheral T-cell lymphoma
Peripheral T-cell lymphomas are uncommon aggressive mature T-cell lymphomas that are operationally modeled here as the nodal and systemic PTCL family, with PTCL-NOS, nodal TFH lymphomas, and systemic ALCL represented as flat subtype facets.
CASE_DEFINITION Disease-level family definition aligned to current nodal and systemic PTCL practice and used as the curation unit for this dismech page.
Show evidence (2 references)
PMID:36010351 SUPPORT Human Clinical
"Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
This review provides the family-level definition for PTCL as an uncommon mature T-cell lymphoma group.
PMID:36010351 SUPPORT Human Clinical
"According to their presentation, PTCLs can be divided into nodal, extranodal or cutaneous, and leukemic types."
This supports modeling PTCL as a family-level curation unit with flat subtype facets rather than a single narrow ontology subclass.
◆

Subtypes

6
Peripheral T-Cell Lymphoma, Not Otherwise Specified MONDO:0004964
Default nodal and systemic PTCL category used when a mature T-cell lymphoma does not meet criteria for TFH lymphoma or systemic ALCL. PTCL-NOS is biologically heterogeneous and includes clinically important GATA3 and TBX21 transcriptional programs.
Show evidence (2 references)
PMID:36010351 SUPPORT Human Clinical
"Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
This supports PTCL-NOS as a core subtype within the nodal and systemic PTCL family.
PMID:31562134 SUPPORT Human Clinical
"Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of mature T-cell malignancies; approximately one-third of cases are designated as PTCL-not otherwise specified (PTCL-NOS)."
This supports PTCL-NOS as a common and clinically meaningful PTCL subtype.
Follicular Helper T-Cell Lymphoma, Angioimmunoblastic-Type MONDO:0004977
Nodal TFH lymphoma subtype with angioimmunoblastic morphology, frequent epigenetic modifier mutations, and prominent immune-microenvironmental remodeling.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This review defines angioimmunoblastic-type TFH lymphoma as one of the three major nodal TFH lymphoma subtypes.
Nodal T-Follicular Helper Cell Lymphoma, Follicular Type MONDO:0958095
Nodal TFH lymphoma subtype with follicular architecture and the TFH-lineage mutational profile shared across the nodal TFH lymphoma spectrum.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This review defines follicular-type TFH lymphoma as one of the three major nodal TFH lymphoma subtypes.
Follicular Helper T-Cell Lymphoma, Not Otherwise Specified
Nodal TFH lymphoma subtype that shows TFH immunophenotypic and molecular features but lacks the defining architecture of the angioimmunoblastic-type or follicular-type entities.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This review defines TFH lymphoma, NOS as one of the three major nodal TFH lymphoma subtypes.
ALK-Positive Anaplastic Large Cell Lymphoma MONDO:0017602
Systemic ALCL subtype driven by ALK rearrangement and generally associated with better outcomes than most other PTCL entities.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
This supports ALK-positive ALCL as a core nodal and systemic PTCL subtype.
ALK-Negative Anaplastic Large Cell Lymphoma MONDO:0017603
Systemic ALCL subtype lacking ALK rearrangement and showing greater genetic heterogeneity than ALK-positive ALCL.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
This supports ALK-negative ALCL as a core nodal and systemic PTCL subtype.
⚙

Pathophysiology

8
TFH-lineage epigenetic deregulation
A major PTCL branch consists of nodal TFH lymphomas in which recurrent mutations in epigenetic regulators distort chromatin state and stabilize malignant TFH-cell identity.
T follicular helper cell CL:0002038 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves T follicular helper cell (CL:0002038). CL:0002038 is a cell type from the Cell Ontology.
chromatin organization GO:0006325 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves dysregulated chromatin organization (GO:0006325). GO:0006325 is a biological process from the Gene Ontology. ↕ DYSREGULATED
Show evidence (2 references)
PMID:36793612 SUPPORT Human Clinical
"These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes."
This supports epigenetic deregulation as a defining mechanistic branch of nodal TFH lymphomas within the PTCL family.
PMID:37841428 SUPPORT Human Clinical
"Mutations in the epigenetic regulator TET2 are among the most frequent mutations identified in PTCL, with the highest frequency in angioimmunoblastic T cell lymphomas and other nodal T follicular helper (TFH) lymphomas."
This supports TET2-centered epigenetic disruption as especially prominent in TFH-derived PTCL.
TFH Angioimmunoblastic-Type RHOA-VAV1-mediated T-cell receptor signaling activation
In angioimmunoblastic-type TFH lymphoma, hotspot RHOA lesions can rewire VAV1 adaptor signaling and accelerate downstream T-cell receptor signaling.
T follicular helper cell CL:0002038 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves T follicular helper cell (CL:0002038). CL:0002038 is a cell type from the Cell Ontology.
T cell receptor signaling pathway GO:0050852 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased T cell receptor signaling pathway (GO:0050852). GO:0050852 is a biological process from the Gene Ontology. ↑ INCREASED
Downstream
TFH Angioimmunoblastic-Type cytokine and chemokine program induction G17V RHOA expression is accompanied by activation of cytokine and chemokine pathway programs.
Show evidence (1 reference)
PMID:28832024 SUPPORT In Vitro
"We found that binding of G17V RHOA to VAV1 augmented its adaptor function through phosphorylation of 174Tyr, resulting in acceleration of T-cell receptor (TCR) signaling."
This mechanistic study directly supports RHOA-VAV1-mediated acceleration of T-cell receptor signaling in angioimmunoblastic-type TFH lymphoma.
TFH Angioimmunoblastic-Type cytokine and chemokine program induction
Aberrant TFH-cell signaling promotes a cytokine- and chemokine-rich program that contributes to the inflammatory and immune-remodeling features of angioimmunoblastic-type PTCL.
positive regulation of cytokine production GO:0001819 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased positive regulation of cytokine production (GO:0001819). GO:0001819 is a biological process from the Gene Ontology. ↑ INCREASED
Show evidence (1 reference)
PMID:28832024 SUPPORT In Vitro
"Enrichment of cytokine and chemokine-related pathways was also evident by the expression of G17V RHOA."
This supports cytokine and chemokine program induction as a distinct downstream mechanism in angioimmunoblastic-type TFH lymphoma.
PTCL-NOS transcriptional polarization
PTCL-NOS separates into reproducible GATA3 and TBX21 programs with distinct biology and prognosis rather than representing a single uniform disease state.
regulation of T cell differentiation GO:0045580 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves dysregulated regulation of T cell differentiation (GO:0045580). GO:0045580 is a biological process from the Gene Ontology. ↕ DYSREGULATED
Downstream
PTCL-NOS GATA3 oncogenic pathway activation One PTCL-NOS branch is GATA3-skewed and genetically complex.
PTCL-NOS DNMT3A-linked cytotoxic program Another PTCL-NOS branch is TBX21/cytotoxic and enriched for epigenetic dysregulation.
Show evidence (1 reference)
PMID:31562134 SUPPORT Human Clinical
"Using gene-expression profiling (GEP), we have previously defined 2 major molecular subtypes of PTCL-NOS, PTCL-GATA3 and PTCL-TBX21, which have distinct biological differences in oncogenic pathways and prognosis."
This supports PTCL-NOS as a mechanistically polarized subtype with at least two major transcriptional programs.
PTCL-NOS GATA3 oncogenic pathway activation
The GATA3-polarized PTCL-NOS program is marked by genomic complexity with lesions converging on tumor-suppressor loss, PI3K pathway dysregulation, and JAK/STAT-MYC signaling.
cell surface receptor signaling pathway via JAK-STAT GO:0007259 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased cell surface receptor signaling pathway via JAK-STAT (GO:0007259). GO:0007259 is a biological process from the Gene Ontology. ↑ INCREASED positive regulation of cell population proliferation GO:0008284 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased positive regulation of cell population proliferation (GO:0008284). GO:0008284 is a biological process from the Gene Ontology. ↑ INCREASED
Show evidence (1 reference)
PMID:30782609 SUPPORT Human Clinical
"PTCL-GATA3 exhibited greater genomic complexity that was characterized by frequent loss or mutation of tumor suppressor genes targeting the CDKN2A /B-TP53 axis and PTEN-PI3K pathways. Co-occurring gains/amplifications of STAT3 and MYC occurred in PTCL-GATA3."
This supports a distinct PTCL-GATA3 oncogenic program centered on proliferative and receptor-signaling pathway lesions.
PTCL-NOS DNMT3A-linked cytotoxic program
A DNMT3A-mutant PTCL-TBX21 branch shows epigenetically rewired cytotoxic T-cell programs coupled to interferon-gamma and T-cell receptor signaling genes.
CD8-positive, alpha-beta cytotoxic T cell CL:0000794 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves CD8-positive, alpha-beta cytotoxic T cell (CL:0000794). CL:0000794 is a cell type from the Cell Ontology.
chromatin organization GO:0006325 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves dysregulated chromatin organization (GO:0006325). GO:0006325 is a biological process from the Gene Ontology. ↕ DYSREGULATED regulation of T cell differentiation GO:0045580 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves dysregulated regulation of T cell differentiation (GO:0045580). GO:0045580 is a biological process from the Gene Ontology. ↕ DYSREGULATED
Show evidence (1 reference)
PMID:35639959 SUPPORT Human Clinical
"Genomewide methylation analysis of DNMT3A-mutant vs wild-type (WT) PTCL-TBX21 cases demonstrated hypomethylation in target genes regulating interferon-γ (IFN-γ), T-cell receptor signaling, and EOMES (eomesodermin), a master transcriptional regulator of cytotoxic effector cells."
This supports a DNMT3A-linked epigenetic and cytotoxic differentiation program within the PTCL-NOS branch.
Immune-checkpoint-rich tumor microenvironment
Nodal PTCL frequently develops in a suppressive immune microenvironment enriched for regulatory T cells, exhausted CD8-positive T cells, and druggable checkpoint programs.
regulatory T cell CL:0000815 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves regulatory T cell (CL:0000815). CL:0000815 is a cell type from the Cell Ontology. exhausted T cell CL:0011025 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves exhausted T cell (CL:0011025). CL:0011025 is a cell type from the Cell Ontology.
negative regulation of immune response GO:0050777 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased negative regulation of immune response (GO:0050777). GO:0050777 is a biological process from the Gene Ontology. ↑ INCREASED
Show evidence (1 reference)
PMID:38813724 SUPPORT Human Clinical
"The PTCL TME contained a larger proportion of regulatory T cells and exhausted CD8+ T cells, with enriched expression of druggable immune checkpoints."
This supports a suppressive, checkpoint-rich tumor microenvironment as a distinct pathophysiologic feature of nodal PTCL.
CAR-T-derived secondary lymphoproliferation via BLM and TET2-mediated transformation
Secondary PTCL can arise clonally from infused CAR-T products in patients with germline or somatic genomic instability. Pre-existing driver mutations (e.g., BLM deficiency) combined with somatic TET2 mutations and CAR vector integration events in cancer-associated genes create a multistep oncogenic process enabling malignant transformation of the therapeutic T-cell product.
CD8-positive, alpha-beta cytotoxic T cell CL:0000794 Cell Ontology (CL) Relation: this pathophysiological event involves this cell type This pathophysiological event involves CD8-positive, alpha-beta cytotoxic T cell (CL:0000794). CL:0000794 is a cell type from the Cell Ontology.
chromatin organization GO:0006325 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves dysregulated chromatin organization (GO:0006325). GO:0006325 is a biological process from the Gene Ontology. ↕ DYSREGULATED positive regulation of cell population proliferation GO:0008284 Gene Ontology (GO) Relation: this pathophysiological event involves this biological process This pathophysiological event involves increased positive regulation of cell population proliferation (GO:0008284). GO:0008284 is a biological process from the Gene Ontology. ↑ INCREASED
Show evidence (3 references)
PMID:42419880 SUPPORT Human Clinical
"We describe a case of secondary peripheral T-cell lymphoma (PTCL) arising from infused CD19/CD22-4-1BB-CD3ζ-lenti bispecific chimeric antigen receptor (CAR)-T cells in a patient with relapsed or refractory B-cell lymphoma who carried a pathogenic germline BLM p.L107Ffs*36 variant."
This case report documents secondary PTCL arising directly from the infused CAR-T product in the context of germline BLM mutation, identifying a novel iatrogenic mechanism of lymphoproliferative transformation.
PMID:42419880 SUPPORT Human Clinical
"Integrated genomic and molecular analyses confirmed that the PTCL was clonally derived from the infused CAR-T product and revealed a multistep oncogenic process involving pre-existing driver mutations (BLM), somatic TET2 mutations (p.R1261H and p.E1755*), and genomic instability."
This integrated genomic analysis supports a multistep model combining germline BLM deficiency, somatic TET2 mutations, and genomic instability as drivers of CAR-T-derived PTCL.
PMID:42419880 SUPPORT Human Clinical
"Clonal CAR vector integration events in cancer-associated genes (NF1, CBX5, RNF213, etc) were identified as markers of clonal expansion, although no functional evidence supports a direct oncogenic role."
CAR vector integration into cancer-associated genomic loci serves as a clonal expansion marker and potential contributor to malignant transformation.
✶

Histopathology

7
PTCL family morphology
Family-level NCIT anchor for the mature T-cell lymphoma spectrum curated on this page.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
This supports the family-level histopathologic placement of PTCL among mature T-cell lymphomas.
PTCL-NOS architecture effacement
PTCL-NOS typically shows paracortical or diffuse infiltrates with architectural effacement and a variable inflammatory background.
Show evidence (1 reference)
PMID:20702104 SUPPORT Human Clinical
"In the lymph node, PTCL-NOS shows paracortical or diffuse infiltrates with effacement of the normal architecture, with a broad cytological spectrum and a frequently observed inflammatory background."
This directly supports the characteristic PTCL-NOS nodal morphology.
TFH lymphoma, angioimmunoblastic-type
NCIT subtype anchor for angioimmunoblastic-type nodal TFH lymphoma.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This supports the angioimmunoblastic-type TFH lymphoma subtype assignment.
TFH lymphoma, follicular-type
NCIT subtype anchor for follicular-type nodal TFH lymphoma.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This supports the follicular-type TFH lymphoma subtype assignment.
TFH lymphoma, NOS
NCIT subtype anchor for TFH lymphoma, NOS.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
This supports the TFH lymphoma, NOS subtype assignment.
Systemic ALCL, ALK-positive
NCIT subtype anchor for systemic ALK-positive ALCL within the PTCL family.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
This supports ALK-positive systemic ALCL as a core PTCL subtype.
Systemic ALCL, ALK-negative
NCIT subtype anchor for systemic ALK-negative ALCL within the PTCL family.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
This supports ALK-negative systemic ALCL as a core PTCL subtype.
⬡

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Peripheral T-Cell Lymphoma Interactive directed graph showing how pathophysiology mechanisms, phenotypes, genetic factors and variants, experimental models, environmental triggers, and treatments relate through causal and linked edges.
●

Phenotypes

6
Blood 1
Anemia HP:0001903 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Anemia (HP:0001903). HP:0001903 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:29302559 SUPPORT Human Clinical
"Common laboratory tests reveal that patients have anemia, thrombocytosis, lymphocytosis, eosinophilia, hypergammaglobulinemia, or increased lactate dehydrogenase."
This review abstract explicitly identifies anemia as a common PTCL-NOS laboratory abnormality.
Cardiovascular 2
Lymphadenopathy HP:0002716 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Lymphadenopathy (HP:0002716). HP:0002716 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"The most frequent primary sites of PTCLs are lymph nodes, with over half of cases showing nodal presentation."
This supports lymph node enlargement as the dominant clinical presentation across nodal and systemic PTCL.
Splenomegaly HP:0001744 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Splenomegaly (HP:0001744). HP:0001744 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:29302559 SUPPORT Human Clinical
"Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
This review abstract explicitly lists splenomegaly among the common PTCL-NOS clinical features.
Metabolism 1
Fever HP:0001945 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Fever (HP:0001945). HP:0001945 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:29302559 SUPPORT Human Clinical
"Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
This review abstract explicitly lists fever among the common PTCL-NOS clinical features.
Constitutional 1
Night Sweats HP:0030166 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Night sweats (HP:0030166). HP:0030166 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:29302559 SUPPORT Human Clinical
"Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
This review abstract explicitly lists night sweats among the common PTCL-NOS clinical features.
Growth 1
Weight Loss HP:0001824 Human Phenotype Ontology (HP) Relation: this clinical feature is this phenotype This clinical feature is Weight loss (HP:0001824). HP:0001824 is a phenotype from the Human Phenotype Ontology.
Show evidence (1 reference)
PMID:29302559 SUPPORT Human Clinical
"Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
This review abstract explicitly lists weight loss among the common PTCL-NOS clinical features.
🧬

Genetic Associations

7
TET2 (Recurrent Somatic Mutation)
Gene: TET2 hgnc:25941 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is TET2 (hgnc:25941). hgnc:25941 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:37841428 SUPPORT Human Clinical
"Mutations in the epigenetic regulator TET2 are among the most frequent mutations identified in PTCL, with the highest frequency in angioimmunoblastic T cell lymphomas and other nodal T follicular helper (TFH) lymphomas."
This supports TET2 as a recurrent, TFH-enriched somatic lesion in PTCL.
DNMT3A (Recurrent Somatic Mutation)
Gene: DNMT3A hgnc:2978 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is DNMT3A (hgnc:2978). hgnc:2978 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:35639959 SUPPORT Human Clinical
"DNMT3A-mutated PTCL-TBX21 cases showed inferior overall survival (OS), with DNMT3A-mutated residues skewed toward the methyltransferase domain and dimerization motif (S881-R887)."
This supports DNMT3A mutation as a defining lesion of a prognostically distinct PTCL-NOS subgroup.
IDH2 (Recurrent Somatic Mutation)
Gene: IDH2 hgnc:5383 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is IDH2 (hgnc:5383). hgnc:5383 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:36793612 SUPPORT Human Clinical
"These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes."
This supports IDH2 as a recurrent component of the nodal TFH lymphoma mutational program.
RHOA (Hotspot G17V Mutation)
Gene: RHOA hgnc:667 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is RHOA (hgnc:667). hgnc:667 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:28832024 SUPPORT Human Clinical
"Somatic G17V RHOA mutations were found in 50-70% of angioimmunoblastic T-cell lymphoma (AITL)."
This supports RHOA G17V as a recurrent hotspot mutation in the angioimmunoblastic PTCL branch.
VAV1 (Activating Mutation or Fusion)
Gene: VAV1 hgnc:12657 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is VAV1 (hgnc:12657). hgnc:12657 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:28832024 SUPPORT Human Clinical
"We further identified VAV1 mutations and a new translocation, VAV1-STAP2, in seven of the 85 RHOA mutation-negative samples (8.2%), whereas none of the 41 RHOA mutation-positive samples exhibited VAV1 mutations."
This supports VAV1 mutations and fusions as alternative TCR-pathway lesions in angioimmunoblastic-type PTCL.
STAT3 (Copy Number Gain / Pathway Activation)
Gene: STAT3 hgnc:11364 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is STAT3 (hgnc:11364). hgnc:11364 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:30782609 SUPPORT Human Clinical
"Co-occurring gains/amplifications of STAT3 and MYC occurred in PTCL-GATA3."
This supports STAT3 pathway activation as a recurrent feature of the PTCL-GATA3 PTCL-NOS program.
BLM (Germline Predisposing Mutation (Bloom Syndrome))
Gene: BLM hgnc:1058 HUGO Gene Nomenclature Committee (hgnc) Relation: this disease-associated gene is this gene This disease-associated gene is BLM (hgnc:1058). hgnc:1058 is a gene from the HUGO Gene Nomenclature Committee.
Show evidence (1 reference)
PMID:42419880 SUPPORT Human Clinical
"We describe a case of secondary peripheral T-cell lymphoma (PTCL) arising from infused CD19/CD22-4-1BB-CD3ζ-lenti bispecific chimeric antigen receptor (CAR)-T cells in a patient with relapsed or refractory B-cell lymphoma who carried a pathogenic germline BLM p.L107Ffs*36 variant."
This case demonstrates germline BLM mutation as a predisposing factor for secondary PTCL transformation of CAR-T products.
💊

Medical Actions

6
CHOP-based Multiagent Chemotherapy
Action: chemotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is chemotherapy (NCIT:C15632). NCIT:C15632 is a clinical intervention from the NCI Thesaurus. Ontology label: Chemotherapy NCIT:C15632 Regimen: CHOP regimenNCI Thesaurus (NCIT) Relation: this regimen component is this clinical intervention This regimen component is CHOP regimen (NCIT:C9549). NCIT:C9549 is a clinical intervention from the NCI Thesaurus. Ontology label: CHOP Regimen NCIT:C9549
Anthracycline-based combination chemotherapy remains the historic frontline backbone for many PTCL cases, although durability is limited.
Mechanism Target:
INHIBITS TFH Angioimmunoblastic-Type cytokine and chemokine program induction — Cytotoxic CHOP-based agents eliminate proliferating malignant TFH and PTCL-NOS cells, suppressing the tumor-driven cytokine and chemokine programs that sustain the immunosuppressive microenvironment.
Show evidence (1 reference)
PMID:30914464 SUPPORT Human Clinical
"Improvement in progression-free and overall survival over cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, which has been the standard of care for decades, is unprecedented."
This approval summary confirms CHOP-like anthracycline chemotherapy as the longstanding frontline standard for PTCL.
Brentuximab Vedotin Plus CHP
Action: chemotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is chemotherapy (NCIT:C15632). NCIT:C15632 is a clinical intervention from the NCI Thesaurus. Ontology label: Chemotherapy NCIT:C15632
Agent: brentuximab vedotin NCIT:C66944 NCI Thesaurus (NCIT) Relation: this treatment uses this therapeutic agent This treatment uses brentuximab vedotin (NCIT:C66944). NCIT:C66944 is a therapeutic agent from the NCI Thesaurus.
Frontline regimen for CD30-expressing PTCL, especially systemic ALCL and selected PTCL-NOS and TFH cases with CD30 expression.
Mechanism Target:
INHIBITS TFH Angioimmunoblastic-Type cytokine and chemokine program induction — Brentuximab vedotin delivers MMAE cytotoxin to CD30-expressing tumor T cells, eliminating the cells that drive the pathological cytokine and chemokine program.
INHIBITS PTCL-NOS GATA3 oncogenic pathway activation — In GATA3+ PTCL-NOS cases with CD30 expression, BV targets the tumor cells driven by the GATA3 oncogenic pathway.
Show evidence (2 references)
PMID:30914464 SUPPORT Human Clinical
"The median PFS was 48.2 months with BV+CHP versus 20.8 months with CHOP"
This directly supports improved frontline progression-free survival with brentuximab vedotin plus CHP in CD30-expressing PTCL.
PMID:30914464 SUPPORT Human Clinical
"The trial also demonstrated improvement in overall survival (HR 0.66; 95% CI: 0.46-0.95), complete response rate (68% vs. 56%), and overall response rate (83% vs. 72%) with BV+CHP."
This supports a broad frontline efficacy advantage for BV+CHP over CHOP.
Pralatrexate
Action: PharmacotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is Pharmacotherapy (NCIT:C15986). NCIT:C15986 is a clinical intervention from the NCI Thesaurus. NCIT:C15986
Agent: pralatrexate NCIT:C2250 NCI Thesaurus (NCIT) Relation: this treatment uses this therapeutic agent This treatment uses pralatrexate (NCIT:C2250). NCIT:C2250 is a therapeutic agent from the NCI Thesaurus.
Antifolate therapy used in relapsed or refractory PTCL after prior systemic treatment.
Mechanism Target:
INHIBITS TFH Angioimmunoblastic-Type cytokine and chemokine program induction — Pralatrexate inhibits dihydrofolate reductase in actively proliferating PTCL tumor cells, blocking DNA synthesis and eliminating the cells sustaining the pathological cytokine program.
Show evidence (1 reference)
PMID:21245435 SUPPORT Human Clinical
"The response rate in 109 evaluable patients was 29% (32 of 109), including 12 complete responses (11%) and 20 partial responses (18%), with a median DoR of 10.1 months."
This pivotal study supports pralatrexate as an active salvage therapy in relapsed or refractory PTCL.
Romidepsin
Action: PharmacotherapyNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is Pharmacotherapy (NCIT:C15986). NCIT:C15986 is a clinical intervention from the NCI Thesaurus. NCIT:C15986
Agent: romidepsin NCIT:C1544 NCI Thesaurus (NCIT) Relation: this treatment uses this therapeutic agent This treatment uses romidepsin (NCIT:C1544). NCIT:C1544 is a therapeutic agent from the NCI Thesaurus.
Histone deacetylase inhibitor with single-agent activity in relapsed PTCL.
Mechanism Target:
INHIBITS TFH-lineage epigenetic deregulation — Romidepsin inhibits histone deacetylases, restoring histone acetylation patterns altered by TET2, DNMT3A, and IDH2 mutations that drive TFH- lineage epigenetic reprogramming in PTCL.
Show evidence (2 references)
PMID:21355097 SUPPORT Human Clinical
"Complete responses were observed in 8 and partial responses in 9 of 45 patients, for an overall response rate of 38% (95% confidence interval 24%-53%)."
This phase 2 study supports romidepsin as an active salvage therapy in relapsed PTCL.
PMID:21355097 SUPPORT Human Clinical
"The histone deacetylase inhibitor romidepsin has single agent clinical activity associated with durable responses in patients with relapsed PTCL."
This supports the durability of romidepsin responses in relapsed PTCL.
Autologous Hematopoietic Stem Cell Transplantation
Action: autologous hematopoietic stem cell transplantationNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is autologous hematopoietic stem cell transplantation (NCIT:C16039). NCIT:C16039 is a clinical intervention from the NCI Thesaurus. Ontology label: Autologous Hematopoietic Stem Cell Transplantation NCIT:C16039
Consolidative transplant strategy considered in fit patients with responsive disease, particularly in first remission or chemosensitive relapse.
Mechanism Target:
INHIBITS TFH Angioimmunoblastic-Type cytokine and chemokine program induction — Myeloablative conditioning eradicates residual chemosensitive PTCL cells that sustain the pathological cytokine program, consolidating remission achieved with induction therapy.
Show evidence (1 reference)
PMID:20359581 SUPPORT Human Clinical
"Studies show that autologous transplant is feasible in relapsed and previously untreated patients, and efficacy is comparable to results in aggressive B-cell lymphomas."
This review supports autologous transplantation as a feasible PTCL consolidation strategy.
Allogeneic Hematopoietic Stem Cell Transplantation
Action: allogeneic hematopoietic stem cell transplantationNCI Thesaurus (NCIT) Relation: this treatment is this clinical intervention This treatment is allogeneic hematopoietic stem cell transplantation (NCIT:C46089). NCIT:C46089 is a clinical intervention from the NCI Thesaurus. Ontology label: Allogeneic Hematopoietic Stem Cell Transplantation NCIT:C46089
Allogeneic transplantation remains an option for selected relapsed PTCL cases, particularly when graft-versus-lymphoma activity is sought.
Mechanism Target:
INHIBITS Immune-checkpoint-rich tumor microenvironment — Allogeneic graft-versus-lymphoma immune surveillance can overcome the immunosuppressive PTCL microenvironment, with donor T cells targeting residual tumor cells.
Show evidence (1 reference)
PMID:20359581 SUPPORT Human Clinical
"Allogeneic transplant may also have a role in relapsed PTCL, especially in the context of reduced-intensity conditioning, which has decreased nonrelapse mortality."
This review supports an allogeneic transplant option in selected relapsed PTCL.
🔬

Biochemical Markers

3
TFH Immunophenotype
Aberrant Pan-T-cell Antigen Loss
CD30 Expression
📈

Progression

2
Diagnosis
Age: Adult to elderly
PTCL predominantly presents in adults and older patients and usually has an aggressive clinical course.
Show evidence (1 reference)
PMID:36010351 SUPPORT Human Clinical
"They predominantly affect adults and elderly people and usually exhibit an aggressive clinical course with poor prognosis."
This supports the typical adult-to-elderly age at presentation and the aggressive behavior of PTCL.
Front-line outcome
Durable control is possible, but most PTCL-related deaths occur within 5 years of diagnosis.
Show evidence (2 references)
PMID:38532575 SUPPORT Human Clinical
"The overall response rate to first-line therapy was 68%, while 5- and 10-year overall survival estimates were 49% and 40% respectively."
This prospective cohort provides the long-term outcome benchmark for the PTCL family.
PMID:38532575 SUPPORT Human Clinical
"Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%."
This supports the front-loaded mortality pattern of PTCL despite a subset of patients achieving durable disease control.
📊

Prevalence

1
All non-Hodgkin lymphomas in Western countries
10
Approximate relative share of PTCL among Western non-Hodgkin lymphomas.
Show evidence (1 reference)
PMID:21138864 SUPPORT Human Clinical
"Peripheral T-cell lymphomas (PTCL) constitute a group of heterogeneous diseases that are uncommon, representing, in Western countries, only approximately 10% of all non-Hodgkin lymphomas."
This review provides a commonly cited family-level prevalence estimate for PTCL in Western countries.
📊

Related Datasets

2
Whole exome sequencing of peripheral T-cell lymphoma (PTCL) ega:EGAS00001000557
Somatic mutations in 3 AITL and 3 PTCL, not otherwise specified (PTCL-NOS) specimens were explored using whole-exome sequencing.
human
European Genome-phenome Archive study, matched because the disease is named in the study's own title ("Peripheral T-Cell Lymphoma"); description-level mentions were not accepted. EGA study_type: Other. Controlled access -- data require a Data Access Agreement. EGA metadata retrieved 2026-08-01.
Genetic and Microenvironmental Analysis of Peripheral T-cell Lymphoma ega:EGAS50000001258
This study enrolled 129 patients diagnosed with PTCL, comprising 94 cases of nTFHL and 35 cases of PTCL-NOS. Whole exome sequencing (WES) and RNA sequencing (RNA-seq) were performed using DNA and RNA of sufficient quality extracted from their tumor specimens. WES was conducted for all 129 cases, while RNA-seq was performed on 57 tumor samples.
human WES
European Genome-phenome Archive study, matched because the disease is named in the study's own title ("Peripheral T-Cell Lymphoma"); description-level mentions were not accepted. EGA study_type: Exome Sequencing. Controlled access -- data require a Data Access Agreement. EGA metadata retrieved 2026-08-01.
{ }

Source YAML

click to show 
name: Peripheral T-Cell Lymphoma
creation_date: '2026-04-13T05:36:37Z'
synonyms:
- PTCL
- peripheral T-cell lymphoma
description: >-
  Peripheral T-cell lymphoma (PTCL) is a clinically used family of aggressive
  mature T-cell non-Hodgkin lymphomas dominated in nodal and systemic practice
  by PTCL-not otherwise specified (PTCL-NOS), nodal T-follicular helper (TFH)
  lymphomas, and systemic anaplastic large cell lymphomas (ALCL). PTCL biology
  is heterogeneous, with subtype-weighted contributions from TFH-lineage
  epigenetic lesions, RHOA/VAV1-mediated T-cell receptor signaling, PTCL-NOS
  GATA3/TBX21 transcriptional programs, and an immune microenvironment enriched
  in regulatory and exhausted T cells. Following the cancer-modeling guidance
  from dismech issue  # 1198, this entry treats PTCL as a single mechanism-graph
  unit and keeps clinically meaningful histologic entities as flat subtype facets
  rather than separate dismech pages. Because MONDO lacks an exact family-level
  peripheral T-cell lymphoma umbrella term, the page is anchored to the closest
  MONDO family class and refined with subtype-specific NCIT histopathology and
  oncology treatment terms.
definitions:
- name: Family-level clinical definition of peripheral T-cell lymphoma
  definition_type: CASE_DEFINITION
  description: >-
    Peripheral T-cell lymphomas are uncommon aggressive mature T-cell lymphomas
    that are operationally modeled here as the nodal and systemic PTCL family,
    with PTCL-NOS, nodal TFH lymphomas, and systemic ALCL represented as flat
    subtype facets.
  scope: >-
    Disease-level family definition aligned to current nodal and systemic PTCL
    practice and used as the curation unit for this dismech page.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
    explanation: >-
      This review provides the family-level definition for PTCL as an uncommon
      mature T-cell lymphoma group.
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "According to their presentation, PTCLs can be divided into nodal, extranodal or cutaneous, and leukemic types."
    explanation: >-
      This supports modeling PTCL as a family-level curation unit with flat
      subtype facets rather than a single narrow ontology subclass.
categories:
- Hematologic Malignancy
- T-cell lymphoma
- Non-Hodgkin Lymphoma
parents:
- T-cell lymphoma
disease_term:
  preferred_term: peripheral T-cell lymphoma
  term:
    id: MONDO:0015760
    label: T-cell non-Hodgkin lymphoma
has_subtypes:
- name: PTCL-NOS
  display_name: Peripheral T-Cell Lymphoma, Not Otherwise Specified
  subtype_term:
    preferred_term: peripheral T-cell lymphoma, not otherwise specified
    term:
      id: MONDO:0004964
      label: peripheral T-cell lymphoma, not otherwise specified
  description: >-
    Default nodal and systemic PTCL category used when a mature T-cell lymphoma
    does not meet criteria for TFH lymphoma or systemic ALCL. PTCL-NOS is
    biologically heterogeneous and includes clinically important GATA3 and TBX21
    transcriptional programs.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
    explanation: >-
      This supports PTCL-NOS as a core subtype within the nodal and systemic
      PTCL family.
  - reference: PMID:31562134
    reference_title: "Reproducing the molecular subclassification of peripheral T-cell lymphoma-NOS by immunohistochemistry."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of mature T-cell malignancies; approximately one-third of cases are designated as PTCL-not otherwise specified (PTCL-NOS)."
    explanation: >-
      This supports PTCL-NOS as a common and clinically meaningful PTCL subtype.
- name: TFH Angioimmunoblastic-Type
  display_name: Follicular Helper T-Cell Lymphoma, Angioimmunoblastic-Type
  subtype_term:
    preferred_term: angioimmunoblastic T-cell lymphoma
    term:
      id: MONDO:0004977
      label: angioimmunoblastic T-cell lymphoma
  description: >-
    Nodal TFH lymphoma subtype with angioimmunoblastic morphology, frequent
    epigenetic modifier mutations, and prominent immune-microenvironmental
    remodeling.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This review defines angioimmunoblastic-type TFH lymphoma as one of the
      three major nodal TFH lymphoma subtypes.
- name: TFH Follicular-Type
  display_name: Nodal T-Follicular Helper Cell Lymphoma, Follicular Type
  subtype_term:
    preferred_term: nodal T-follicular helper cell lymphoma, follicular type
    term:
      id: MONDO:0958095
      label: Nodal T-follicular helper cell lymphoma, follicular type
  description: >-
    Nodal TFH lymphoma subtype with follicular architecture and the TFH-lineage
    mutational profile shared across the nodal TFH lymphoma spectrum.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This review defines follicular-type TFH lymphoma as one of the three major
      nodal TFH lymphoma subtypes.
- name: TFH NOS
  display_name: Follicular Helper T-Cell Lymphoma, Not Otherwise Specified
  description: >-
    Nodal TFH lymphoma subtype that shows TFH immunophenotypic and molecular
    features but lacks the defining architecture of the angioimmunoblastic-type
    or follicular-type entities.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This review defines TFH lymphoma, NOS as one of the three major nodal TFH
      lymphoma subtypes.
- name: ALK-Positive ALCL
  display_name: ALK-Positive Anaplastic Large Cell Lymphoma
  subtype_term:
    preferred_term: ALK-positive anaplastic large cell lymphoma
    term:
      id: MONDO:0017602
      label: ALK-positive anaplastic large cell lymphoma
  description: >-
    Systemic ALCL subtype driven by ALK rearrangement and generally associated
    with better outcomes than most other PTCL entities.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
    explanation: >-
      This supports ALK-positive ALCL as a core nodal and systemic PTCL subtype.
- name: ALK-Negative ALCL
  display_name: ALK-Negative Anaplastic Large Cell Lymphoma
  subtype_term:
    preferred_term: ALK-negative anaplastic large cell lymphoma
    term:
      id: MONDO:0017603
      label: ALK-negative anaplastic large cell lymphoma
  description: >-
    Systemic ALCL subtype lacking ALK rearrangement and showing greater genetic
    heterogeneity than ALK-positive ALCL.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
    explanation: >-
      This supports ALK-negative ALCL as a core nodal and systemic PTCL subtype.
prevalence:
- population: All non-Hodgkin lymphomas in Western countries
  percentage: 10
  notes: Approximate relative share of PTCL among Western non-Hodgkin lymphomas.
  evidence:
  - reference: PMID:21138864
    reference_title: "New strategies in peripheral T-cell lymphoma: understanding tumor biology and developing novel therapies."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Peripheral T-cell lymphomas (PTCL) constitute a group of heterogeneous diseases that are uncommon, representing, in Western countries, only approximately 10% of all non-Hodgkin lymphomas."
    explanation: >-
      This review provides a commonly cited family-level prevalence estimate for
      PTCL in Western countries.
progression:
- phase: Diagnosis
  age_range: Adult to elderly
  notes: PTCL predominantly presents in adults and older patients and usually has an aggressive clinical course.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "They predominantly affect adults and elderly people and usually exhibit an aggressive clinical course with poor prognosis."
    explanation: >-
      This supports the typical adult-to-elderly age at presentation and the
      aggressive behavior of PTCL.
- phase: Front-line outcome
  notes: Durable control is possible, but most PTCL-related deaths occur within 5 years of diagnosis.
  evidence:
  - reference: PMID:38532575
    reference_title: "Long-term outcome of peripheral T-cell lymphomas: Ten-year follow-up of the International Prospective T-cell Project."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The overall response rate to first-line therapy was 68%, while 5- and 10-year overall survival estimates were 49% and 40% respectively."
    explanation: >-
      This prospective cohort provides the long-term outcome benchmark for the
      PTCL family.
  - reference: PMID:38532575
    reference_title: "Long-term outcome of peripheral T-cell lymphomas: Ten-year follow-up of the International Prospective T-cell Project."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%."
    explanation: >-
      This supports the front-loaded mortality pattern of PTCL despite a subset
      of patients achieving durable disease control.
pathophysiology:
- name: TFH-lineage epigenetic deregulation
  description: >-
    A major PTCL branch consists of nodal TFH lymphomas in which recurrent
    mutations in epigenetic regulators distort chromatin state and stabilize
    malignant TFH-cell identity.
  cell_types:
  - preferred_term: T follicular helper cell
    term:
      id: CL:0002038
      label: T follicular helper cell
  biological_processes:
  - preferred_term: chromatin organization
    modifier: DYSREGULATED
    term:
      id: GO:0006325
      label: chromatin organization
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes."
    explanation: >-
      This supports epigenetic deregulation as a defining mechanistic branch of
      nodal TFH lymphomas within the PTCL family.
  - reference: PMID:37841428
    reference_title: "Biological insights into the role of TET2 in T cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Mutations in the epigenetic regulator TET2 are among the most frequent mutations identified in PTCL, with the highest frequency in angioimmunoblastic T cell lymphomas and other nodal T follicular helper (TFH) lymphomas."
    explanation: >-
      This supports TET2-centered epigenetic disruption as especially prominent
      in TFH-derived PTCL.
- name: TFH Angioimmunoblastic-Type RHOA-VAV1-mediated T-cell receptor signaling activation
  description: >-
    In angioimmunoblastic-type TFH lymphoma, hotspot RHOA lesions can rewire
    VAV1 adaptor signaling and accelerate downstream T-cell receptor signaling.
  cell_types:
  - preferred_term: T follicular helper cell
    term:
      id: CL:0002038
      label: T follicular helper cell
  biological_processes:
  - preferred_term: T cell receptor signaling pathway
    modifier: INCREASED
    term:
      id: GO:0050852
      label: T cell receptor signaling pathway
  evidence:
  - reference: PMID:28832024
    reference_title: "Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "We found that binding of G17V RHOA to VAV1 augmented its adaptor function through phosphorylation of 174Tyr, resulting in acceleration of T-cell receptor (TCR) signaling."
    explanation: >-
      This mechanistic study directly supports RHOA-VAV1-mediated acceleration
      of T-cell receptor signaling in angioimmunoblastic-type TFH lymphoma.
  downstream:
  - target: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
    description: G17V RHOA expression is accompanied by activation of cytokine and chemokine pathway programs.
    evidence:
    - reference: PMID:28832024
      reference_title: "Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma."
      supports: SUPPORT
      evidence_source: IN_VITRO
      snippet: "Enrichment of cytokine and chemokine-related pathways was also evident by the expression of G17V RHOA."
      explanation: >-
        This supports a direct downstream link from aberrant RHOA-VAV1 signaling
        to cytokine and chemokine pathway induction.
- name: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
  description: >-
    Aberrant TFH-cell signaling promotes a cytokine- and chemokine-rich program
    that contributes to the inflammatory and immune-remodeling features of
    angioimmunoblastic-type PTCL.
  biological_processes:
  - preferred_term: positive regulation of cytokine production
    modifier: INCREASED
    term:
      id: GO:0001819
      label: positive regulation of cytokine production
  evidence:
  - reference: PMID:28832024
    reference_title: "Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma."
    supports: SUPPORT
    evidence_source: IN_VITRO
    snippet: "Enrichment of cytokine and chemokine-related pathways was also evident by the expression of G17V RHOA."
    explanation: >-
      This supports cytokine and chemokine program induction as a distinct
      downstream mechanism in angioimmunoblastic-type TFH lymphoma.
- name: PTCL-NOS transcriptional polarization
  description: >-
    PTCL-NOS separates into reproducible GATA3 and TBX21 programs with distinct
    biology and prognosis rather than representing a single uniform disease state.
  biological_processes:
  - preferred_term: regulation of T cell differentiation
    modifier: DYSREGULATED
    term:
      id: GO:0045580
      label: regulation of T cell differentiation
  evidence:
  - reference: PMID:31562134
    reference_title: "Reproducing the molecular subclassification of peripheral T-cell lymphoma-NOS by immunohistochemistry."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Using gene-expression profiling (GEP), we have previously defined 2 major molecular subtypes of PTCL-NOS, PTCL-GATA3 and PTCL-TBX21, which have distinct biological differences in oncogenic pathways and prognosis."
    explanation: >-
      This supports PTCL-NOS as a mechanistically polarized subtype with at least
      two major transcriptional programs.
  downstream:
  - target: PTCL-NOS GATA3 oncogenic pathway activation
    description: One PTCL-NOS branch is GATA3-skewed and genetically complex.
  - target: PTCL-NOS DNMT3A-linked cytotoxic program
    description: Another PTCL-NOS branch is TBX21/cytotoxic and enriched for epigenetic dysregulation.
- name: PTCL-NOS GATA3 oncogenic pathway activation
  description: >-
    The GATA3-polarized PTCL-NOS program is marked by genomic complexity with
    lesions converging on tumor-suppressor loss, PI3K pathway dysregulation, and
    JAK/STAT-MYC signaling.
  biological_processes:
  - preferred_term: cell surface receptor signaling pathway via JAK-STAT
    modifier: INCREASED
    term:
      id: GO:0007259
      label: cell surface receptor signaling pathway via JAK-STAT
  - preferred_term: positive regulation of cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008284
      label: positive regulation of cell population proliferation
  evidence:
  - reference: PMID:30782609
    reference_title: "Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "PTCL-GATA3 exhibited greater genomic complexity that was characterized by frequent loss or mutation of tumor suppressor genes targeting the CDKN2A /B-TP53 axis and PTEN-PI3K pathways. Co-occurring gains/amplifications of STAT3 and MYC occurred in PTCL-GATA3."
    explanation: >-
      This supports a distinct PTCL-GATA3 oncogenic program centered on
      proliferative and receptor-signaling pathway lesions.
- name: PTCL-NOS DNMT3A-linked cytotoxic program
  description: >-
    A DNMT3A-mutant PTCL-TBX21 branch shows epigenetically rewired cytotoxic
    T-cell programs coupled to interferon-gamma and T-cell receptor signaling genes.
  cell_types:
  - preferred_term: CD8-positive, alpha-beta cytotoxic T cell
    term:
      id: CL:0000794
      label: CD8-positive, alpha-beta cytotoxic T cell
  biological_processes:
  - preferred_term: chromatin organization
    modifier: DYSREGULATED
    term:
      id: GO:0006325
      label: chromatin organization
  - preferred_term: regulation of T cell differentiation
    modifier: DYSREGULATED
    term:
      id: GO:0045580
      label: regulation of T cell differentiation
  evidence:
  - reference: PMID:35639959
    reference_title: "DNMT3A mutations define a unique biological and prognostic subgroup associated with cytotoxic T cells in PTCL-NOS."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Genomewide methylation analysis of DNMT3A-mutant vs wild-type (WT) PTCL-TBX21 cases demonstrated hypomethylation in target genes regulating interferon-γ (IFN-γ), T-cell receptor signaling, and EOMES (eomesodermin), a master transcriptional regulator of cytotoxic effector cells."
    explanation: >-
      This supports a DNMT3A-linked epigenetic and cytotoxic differentiation
      program within the PTCL-NOS branch.
- name: Immune-checkpoint-rich tumor microenvironment
  description: >-
    Nodal PTCL frequently develops in a suppressive immune microenvironment
    enriched for regulatory T cells, exhausted CD8-positive T cells, and
    druggable checkpoint programs.
  cell_types:
  - preferred_term: regulatory T cell
    term:
      id: CL:0000815
      label: regulatory T cell
  - preferred_term: exhausted T cell
    term:
      id: CL:0011025
      label: exhausted T cell
  biological_processes:
  - preferred_term: negative regulation of immune response
    modifier: INCREASED
    term:
      id: GO:0050777
      label: negative regulation of immune response
  evidence:
  - reference: PMID:38813724
    reference_title: "Deep phenotyping of nodal T-cell lymphomas reveals immune alterations and therapeutic targets."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The PTCL TME contained a larger proportion of regulatory T cells and exhausted CD8+ T cells, with enriched expression of druggable immune checkpoints."
    explanation: >-
      This supports a suppressive, checkpoint-rich tumor microenvironment as a
      distinct pathophysiologic feature of nodal PTCL.
- name: CAR-T-derived secondary lymphoproliferation via BLM and TET2-mediated transformation
  description: >-
    Secondary PTCL can arise clonally from infused CAR-T products in patients
    with germline or somatic genomic instability. Pre-existing driver mutations
    (e.g., BLM deficiency) combined with somatic TET2 mutations and CAR vector
    integration events in cancer-associated genes create a multistep oncogenic
    process enabling malignant transformation of the therapeutic T-cell product.
  cell_types:
  - preferred_term: CD8-positive, alpha-beta cytotoxic T cell
    term:
      id: CL:0000794
      label: CD8-positive, alpha-beta cytotoxic T cell
  biological_processes:
  - preferred_term: chromatin organization
    modifier: DYSREGULATED
    term:
      id: GO:0006325
      label: chromatin organization
  - preferred_term: positive regulation of cell population proliferation
    modifier: INCREASED
    term:
      id: GO:0008284
      label: positive regulation of cell population proliferation
  evidence:
  - reference: PMID:42419880
    reference_title: "Secondary peripheral T-cell lymphoma in a patient with DLBCL harboring BLM mutation following CD19/CD22 bispecific CAR-T cell therapy."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "We describe a case of secondary peripheral T-cell lymphoma (PTCL) arising from infused CD19/CD22-4-1BB-CD3ζ-lenti bispecific chimeric antigen receptor (CAR)-T cells in a patient with relapsed or refractory B-cell lymphoma who carried a pathogenic germline BLM p.L107Ffs*36 variant."
    explanation: >-
      This case report documents secondary PTCL arising directly from the
      infused CAR-T product in the context of germline BLM mutation, identifying
      a novel iatrogenic mechanism of lymphoproliferative transformation.
  - reference: PMID:42419880
    reference_title: "Secondary peripheral T-cell lymphoma in a patient with DLBCL harboring BLM mutation following CD19/CD22 bispecific CAR-T cell therapy."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Integrated genomic and molecular analyses confirmed that the PTCL was clonally derived from the infused CAR-T product and revealed a multistep oncogenic process involving pre-existing driver mutations (BLM), somatic TET2 mutations (p.R1261H and p.E1755*), and genomic instability."
    explanation: >-
      This integrated genomic analysis supports a multistep model combining
      germline BLM deficiency, somatic TET2 mutations, and genomic instability
      as drivers of CAR-T-derived PTCL.
  - reference: PMID:42419880
    reference_title: "Secondary peripheral T-cell lymphoma in a patient with DLBCL harboring BLM mutation following CD19/CD22 bispecific CAR-T cell therapy."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Clonal CAR vector integration events in cancer-associated genes (NF1, CBX5, RNF213, etc) were identified as markers of clonal expansion, although no functional evidence supports a direct oncogenic role."
    explanation: >-
      CAR vector integration into cancer-associated genomic loci serves as a
      clonal expansion marker and potential contributor to malignant transformation.
histopathology:
- name: PTCL family morphology
  finding_term:
    preferred_term: peripheral T-cell lymphoma family
    term:
      id: NCIT:C3468
      label: Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
  description: >-
    Family-level NCIT anchor for the mature T-cell lymphoma spectrum curated on
    this page.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
    explanation: >-
      This supports the family-level histopathologic placement of PTCL among
      mature T-cell lymphomas.
- name: PTCL-NOS architecture effacement
  subtype: PTCL-NOS
  finding_term:
    preferred_term: peripheral T-cell lymphoma, not otherwise specified
    term:
      id: NCIT:C4340
      label: Peripheral T-Cell Lymphoma, Not Otherwise Specified
  description: >-
    PTCL-NOS typically shows paracortical or diffuse infiltrates with
    architectural effacement and a variable inflammatory background.
  evidence:
  - reference: PMID:20702104
    reference_title: "Peripheral T-cell lymphoma--not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "In the lymph node, PTCL-NOS shows paracortical or diffuse infiltrates with effacement of the normal architecture, with a broad cytological spectrum and a frequently observed inflammatory background."
    explanation: >-
      This directly supports the characteristic PTCL-NOS nodal morphology.
- name: TFH lymphoma, angioimmunoblastic-type
  subtype: TFH Angioimmunoblastic-Type
  finding_term:
    preferred_term: follicular helper T-cell lymphoma, angioimmunoblastic-type
    term:
      id: NCIT:C7528
      label: Follicular Helper T-Cell Lymphoma, Angioimmunoblastic-Type
  description: NCIT subtype anchor for angioimmunoblastic-type nodal TFH lymphoma.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This supports the angioimmunoblastic-type TFH lymphoma subtype assignment.
- name: TFH lymphoma, follicular-type
  subtype: TFH Follicular-Type
  finding_term:
    preferred_term: follicular helper T-cell lymphoma, follicular-type
    term:
      id: NCIT:C80375
      label: Follicular Helper T-Cell Lymphoma, Follicular-Type
  description: NCIT subtype anchor for follicular-type nodal TFH lymphoma.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This supports the follicular-type TFH lymphoma subtype assignment.
- name: TFH lymphoma, NOS
  subtype: TFH NOS
  finding_term:
    preferred_term: follicular helper T-cell lymphoma, not otherwise specified
    term:
      id: NCIT:C139011
      label: Follicular Helper T-Cell Lymphoma, Not Otherwise Specified
  description: NCIT subtype anchor for TFH lymphoma, NOS.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."
    explanation: >-
      This supports the TFH lymphoma, NOS subtype assignment.
- name: Systemic ALCL, ALK-positive
  subtype: ALK-Positive ALCL
  finding_term:
    preferred_term: systemic anaplastic large cell lymphoma, ALK-positive
    term:
      id: NCIT:C37195
      label: Systemic Anaplastic Large Cell Lymphoma, ALK-Positive
  description: NCIT subtype anchor for systemic ALK-positive ALCL within the PTCL family.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
    explanation: >-
      This supports ALK-positive systemic ALCL as a core PTCL subtype.
- name: Systemic ALCL, ALK-negative
  subtype: ALK-Negative ALCL
  finding_term:
    preferred_term: systemic anaplastic large cell lymphoma, ALK-negative
    term:
      id: NCIT:C37196
      label: Systemic Anaplastic Large Cell Lymphoma, ALK-Negative
  description: NCIT subtype anchor for systemic ALK-negative ALCL within the PTCL family.
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
    explanation: >-
      This supports ALK-negative systemic ALCL as a core PTCL subtype.
phenotypes:
- category: Lymphatic
  name: Lymphadenopathy
  description: >-
    Lymph node enlargement is the dominant presenting pattern across the nodal
    and systemic PTCL family.
  phenotype_term:
    preferred_term: Lymphadenopathy
    term:
      id: HP:0002716
      label: Lymphadenopathy
  evidence:
  - reference: PMID:36010351
    reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The most frequent primary sites of PTCLs are lymph nodes, with over half of cases showing nodal presentation."
    explanation: >-
      This supports lymph node enlargement as the dominant clinical presentation
      across nodal and systemic PTCL.
- category: Constitutional
  name: Fever
  subtype: PTCL-NOS
  description: Fever is part of the B-symptom complex frequently reported in PTCL-NOS.
  phenotype_term:
    preferred_term: Fever
    term:
      id: HP:0001945
      label: Fever
  evidence:
  - reference: PMID:29302559
    reference_title: "Peripheral T-cell lymphoma, not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
    explanation: >-
      This review abstract explicitly lists fever among the common PTCL-NOS
      clinical features.
- category: Constitutional
  name: Night Sweats
  subtype: PTCL-NOS
  description: Night sweats are part of the systemic B-symptom complex in PTCL-NOS.
  phenotype_term:
    preferred_term: Night sweats
    term:
      id: HP:0030166
      label: Night sweats
  evidence:
  - reference: PMID:29302559
    reference_title: "Peripheral T-cell lymphoma, not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
    explanation: >-
      This review abstract explicitly lists night sweats among the common
      PTCL-NOS clinical features.
- category: Constitutional
  name: Weight Loss
  subtype: PTCL-NOS
  description: Unintentional weight loss is a common systemic symptom in PTCL-NOS.
  phenotype_term:
    preferred_term: Weight loss
    term:
      id: HP:0001824
      label: Weight loss
  evidence:
  - reference: PMID:29302559
    reference_title: "Peripheral T-cell lymphoma, not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
    explanation: >-
      This review abstract explicitly lists weight loss among the common
      PTCL-NOS clinical features.
- category: Abdominal
  name: Splenomegaly
  subtype: PTCL-NOS
  description: Splenic enlargement can accompany disseminated PTCL-NOS.
  phenotype_term:
    preferred_term: Splenomegaly
    term:
      id: HP:0001744
      label: Splenomegaly
  evidence:
  - reference: PMID:29302559
    reference_title: "Peripheral T-cell lymphoma, not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
    explanation: >-
      This review abstract explicitly lists splenomegaly among the common
      PTCL-NOS clinical features.
- category: Hematologic
  name: Anemia
  subtype: PTCL-NOS
  description: Anemia is a common laboratory abnormality in PTCL-NOS.
  phenotype_term:
    preferred_term: Anemia
    term:
      id: HP:0001903
      label: Anemia
  evidence:
  - reference: PMID:29302559
    reference_title: "Peripheral T-cell lymphoma, not otherwise specified."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Common laboratory tests reveal that patients have anemia, thrombocytosis, lymphocytosis, eosinophilia, hypergammaglobulinemia, or increased lactate dehydrogenase."
    explanation: >-
      This review abstract explicitly identifies anemia as a common PTCL-NOS
      laboratory abnormality.
biochemical:
- name: TFH Immunophenotype
  notes: >-
    Nodal TFH lymphomas are typically identified with a TFH-marker panel that
    includes PD-1, CXCL13, CXCR5, ICOS, BCL6, and CD10.
- name: Aberrant Pan-T-cell Antigen Loss
  notes: >-
    PTCL-NOS frequently shows an aberrant T-cell phenotype with loss of CD5
    and CD7, most often with a CD4-positive phenotype in nodal disease.
- name: CD30 Expression
  notes: >-
    CD30 expression identifies the PTCL subset eligible for frontline
    brentuximab vedotin plus CHP and is especially relevant in systemic ALCL.
genetic:
- name: TET2
  association: Recurrent Somatic Mutation
  subtype: TFH Angioimmunoblastic-Type
  gene_term:
    preferred_term: TET2
    term:
      id: hgnc:25941
      label: TET2
  notes: >-
    TET2 mutations are among the most frequent lesions in PTCL and are
    especially enriched in angioimmunoblastic-type and other nodal TFH
    lymphomas.
  evidence:
  - reference: PMID:37841428
    reference_title: "Biological insights into the role of TET2 in T cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Mutations in the epigenetic regulator TET2 are among the most frequent mutations identified in PTCL, with the highest frequency in angioimmunoblastic T cell lymphomas and other nodal T follicular helper (TFH) lymphomas."
    explanation: >-
      This supports TET2 as a recurrent, TFH-enriched somatic lesion in PTCL.
- name: DNMT3A
  association: Recurrent Somatic Mutation
  subtype: PTCL-NOS
  gene_term:
    preferred_term: DNMT3A
    term:
      id: hgnc:2978
      label: DNMT3A
  notes: >-
    DNMT3A mutations define a biologically distinct PTCL-TBX21 subset within
    PTCL-NOS that is linked to cytotoxic differentiation programs and worse
    outcome.
  evidence:
  - reference: PMID:35639959
    reference_title: "DNMT3A mutations define a unique biological and prognostic subgroup associated with cytotoxic T cells in PTCL-NOS."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "DNMT3A-mutated PTCL-TBX21 cases showed inferior overall survival (OS), with DNMT3A-mutated residues skewed toward the methyltransferase domain and dimerization motif (S881-R887)."
    explanation: >-
      This supports DNMT3A mutation as a defining lesion of a prognostically
      distinct PTCL-NOS subgroup.
- name: IDH2
  association: Recurrent Somatic Mutation
  subtype: TFH Angioimmunoblastic-Type
  gene_term:
    preferred_term: IDH2
    term:
      id: hgnc:5383
      label: IDH2
  notes: >-
    IDH2 mutations are part of the characteristic TFH-lymphoma mutational
    landscape and commonly co-occur with TET2 and DNMT3A lesions.
  evidence:
  - reference: PMID:36793612
    reference_title: "Pathologic and molecular insights in nodal T-follicular helper cell lymphomas."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes."
    explanation: >-
      This supports IDH2 as a recurrent component of the nodal TFH lymphoma
      mutational program.
- name: RHOA
  association: Hotspot G17V Mutation
  subtype: TFH Angioimmunoblastic-Type
  gene_term:
    preferred_term: RHOA
    term:
      id: hgnc:667
      label: RHOA
  notes: >-
    RHOA G17V is a recurrent hotspot lesion in angioimmunoblastic-type PTCL and
    rewires proximal T-cell receptor signaling through VAV1.
  evidence:
  - reference: PMID:28832024
    reference_title: "Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Somatic G17V RHOA mutations were found in 50-70% of angioimmunoblastic T-cell lymphoma (AITL)."
    explanation: >-
      This supports RHOA G17V as a recurrent hotspot mutation in the
      angioimmunoblastic PTCL branch.
- name: VAV1
  association: Activating Mutation or Fusion
  subtype: TFH Angioimmunoblastic-Type
  gene_term:
    preferred_term: VAV1
    term:
      id: hgnc:12657
      label: VAV1
  notes: >-
    VAV1 mutations and fusions define a complementary mechanism for proximal
    T-cell receptor pathway activation in RHOA-wildtype angioimmunoblastic PTCL.
  evidence:
  - reference: PMID:28832024
    reference_title: "Activation of RHOA-VAV1 signaling in angioimmunoblastic T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "We further identified VAV1 mutations and a new translocation, VAV1-STAP2, in seven of the 85 RHOA mutation-negative samples (8.2%), whereas none of the 41 RHOA mutation-positive samples exhibited VAV1 mutations."
    explanation: >-
      This supports VAV1 mutations and fusions as alternative TCR-pathway lesions
      in angioimmunoblastic-type PTCL.
- name: STAT3
  association: Copy Number Gain / Pathway Activation
  subtype: PTCL-NOS
  gene_term:
    preferred_term: STAT3
    term:
      id: hgnc:11364
      label: STAT3
  notes: >-
    PTCL-GATA3 frequently acquires STAT3 gains or amplifications alongside other
    proliferative pathway lesions.
  evidence:
  - reference: PMID:30782609
    reference_title: "Genetic drivers of oncogenic pathways in molecular subgroups of peripheral T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Co-occurring gains/amplifications of STAT3 and MYC occurred in PTCL-GATA3."
    explanation: >-
      This supports STAT3 pathway activation as a recurrent feature of the
      PTCL-GATA3 PTCL-NOS program.
- name: BLM
  association: Germline Predisposing Mutation (Bloom Syndrome)
  gene_term:
    preferred_term: BLM
    term:
      id: hgnc:1058
      label: BLM
  notes: >-
    Germline BLM mutations predispose to genomic instability and clonal evolution,
    and can contribute to secondary PTCL transformation in patients receiving
    CAR-T cell therapy. BLM deficiency combines with somatic TET2 mutations and
    CAR vector integration to enable malignant progression of the therapeutic
    T-cell product.
  evidence:
  - reference: PMID:42419880
    reference_title: "Secondary peripheral T-cell lymphoma in a patient with DLBCL harboring BLM mutation following CD19/CD22 bispecific CAR-T cell therapy."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "We describe a case of secondary peripheral T-cell lymphoma (PTCL) arising from infused CD19/CD22-4-1BB-CD3ζ-lenti bispecific chimeric antigen receptor (CAR)-T cells in a patient with relapsed or refractory B-cell lymphoma who carried a pathogenic germline BLM p.L107Ffs*36 variant."
    explanation: >-
      This case demonstrates germline BLM mutation as a predisposing factor for
      secondary PTCL transformation of CAR-T products.
treatments:
- name: CHOP-based Multiagent Chemotherapy
  description: >-
    Anthracycline-based combination chemotherapy remains the historic frontline
    backbone for many PTCL cases, although durability is limited.
  target_mechanisms:
  - target: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
    treatment_effect: INHIBITS
    description: >-
      Cytotoxic CHOP-based agents eliminate proliferating malignant TFH and
      PTCL-NOS cells, suppressing the tumor-driven cytokine and chemokine
      programs that sustain the immunosuppressive microenvironment.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: NCIT:C15632
      label: Chemotherapy
  regimen_term:
    preferred_term: CHOP regimen
    term:
      id: NCIT:C9549
      label: CHOP Regimen
  evidence:
  - reference: PMID:30914464
    reference_title: "FDA Approval Summary: Brentuximab Vedotin in First-Line Treatment of Peripheral T-Cell Lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Improvement in progression-free and overall survival over cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, which has been the standard of care for decades, is unprecedented."
    explanation: >-
      This approval summary confirms CHOP-like anthracycline chemotherapy as the
      longstanding frontline standard for PTCL.
- name: Brentuximab Vedotin Plus CHP
  description: >-
    Frontline regimen for CD30-expressing PTCL, especially systemic ALCL and
    selected PTCL-NOS and TFH cases with CD30 expression.
  target_mechanisms:
  - target: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
    treatment_effect: INHIBITS
    description: >-
      Brentuximab vedotin delivers MMAE cytotoxin to CD30-expressing tumor T
      cells, eliminating the cells that drive the pathological cytokine and
      chemokine program.
  - target: PTCL-NOS GATA3 oncogenic pathway activation
    treatment_effect: INHIBITS
    description: >-
      In GATA3+ PTCL-NOS cases with CD30 expression, BV targets the tumor
      cells driven by the GATA3 oncogenic pathway.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: NCIT:C15632
      label: Chemotherapy
    therapeutic_agent:
    - preferred_term: brentuximab vedotin
      term:
        id: NCIT:C66944
        label: Brentuximab Vedotin
  evidence:
  - reference: PMID:30914464
    reference_title: "FDA Approval Summary: Brentuximab Vedotin in First-Line Treatment of Peripheral T-Cell Lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The median PFS was 48.2 months with BV+CHP versus 20.8 months with CHOP"
    explanation: >-
      This directly supports improved frontline progression-free survival with
      brentuximab vedotin plus CHP in CD30-expressing PTCL.
  - reference: PMID:30914464
    reference_title: "FDA Approval Summary: Brentuximab Vedotin in First-Line Treatment of Peripheral T-Cell Lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The trial also demonstrated improvement in overall survival (HR 0.66; 95% CI: 0.46-0.95), complete response rate (68% vs. 56%), and overall response rate (83% vs. 72%) with BV+CHP."
    explanation: >-
      This supports a broad frontline efficacy advantage for BV+CHP over CHOP.
- name: Pralatrexate
  description: >-
    Antifolate therapy used in relapsed or refractory PTCL after prior systemic
    treatment.
  target_mechanisms:
  - target: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
    treatment_effect: INHIBITS
    description: >-
      Pralatrexate inhibits dihydrofolate reductase in actively proliferating
      PTCL tumor cells, blocking DNA synthesis and eliminating the cells
      sustaining the pathological cytokine program.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: pralatrexate
      term:
        id: NCIT:C2250
        label: Pralatrexate
  evidence:
  - reference: PMID:21245435
    reference_title: "Pralatrexate in patients with relapsed or refractory peripheral T-cell lymphoma: results from the pivotal PROPEL study."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The response rate in 109 evaluable patients was 29% (32 of 109), including 12 complete responses (11%) and 20 partial responses (18%), with a median DoR of 10.1 months."
    explanation: >-
      This pivotal study supports pralatrexate as an active salvage therapy in
      relapsed or refractory PTCL.
- name: Romidepsin
  description: >-
    Histone deacetylase inhibitor with single-agent activity in relapsed PTCL.
  target_mechanisms:
  - target: TFH-lineage epigenetic deregulation
    treatment_effect: INHIBITS
    description: >-
      Romidepsin inhibits histone deacetylases, restoring histone acetylation
      patterns altered by TET2, DNMT3A, and IDH2 mutations that drive TFH-
      lineage epigenetic reprogramming in PTCL.
  treatment_term:
    preferred_term: Pharmacotherapy
    term:
      id: NCIT:C15986
      label: Pharmacotherapy
    therapeutic_agent:
    - preferred_term: romidepsin
      term:
        id: NCIT:C1544
        label: Romidepsin
  evidence:
  - reference: PMID:21355097
    reference_title: "Phase 2 trial of romidepsin in patients with peripheral T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Complete responses were observed in 8 and partial responses in 9 of 45 patients, for an overall response rate of 38% (95% confidence interval 24%-53%)."
    explanation: >-
      This phase 2 study supports romidepsin as an active salvage therapy in
      relapsed PTCL.
  - reference: PMID:21355097
    reference_title: "Phase 2 trial of romidepsin in patients with peripheral T-cell lymphoma."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "The histone deacetylase inhibitor romidepsin has single agent clinical activity associated with durable responses in patients with relapsed PTCL."
    explanation: >-
      This supports the durability of romidepsin responses in relapsed PTCL.
- name: Autologous Hematopoietic Stem Cell Transplantation
  description: >-
    Consolidative transplant strategy considered in fit patients with responsive
    disease, particularly in first remission or chemosensitive relapse.
  target_mechanisms:
  - target: TFH Angioimmunoblastic-Type cytokine and chemokine program induction
    treatment_effect: INHIBITS
    description: >-
      Myeloablative conditioning eradicates residual chemosensitive PTCL cells
      that sustain the pathological cytokine program, consolidating remission
      achieved with induction therapy.
  treatment_term:
    preferred_term: autologous hematopoietic stem cell transplantation
    term:
      id: NCIT:C16039
      label: Autologous Hematopoietic Stem Cell Transplantation
  evidence:
  - reference: PMID:20359581
    reference_title: "High-dose therapy and stem cell transplantation."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Studies show that autologous transplant is feasible in relapsed and previously untreated patients, and efficacy is comparable to results in aggressive B-cell lymphomas."
    explanation: >-
      This review supports autologous transplantation as a feasible PTCL
      consolidation strategy.
- name: Allogeneic Hematopoietic Stem Cell Transplantation
  description: >-
    Allogeneic transplantation remains an option for selected relapsed PTCL
    cases, particularly when graft-versus-lymphoma activity is sought.
  target_mechanisms:
  - target: Immune-checkpoint-rich tumor microenvironment
    treatment_effect: INHIBITS
    description: >-
      Allogeneic graft-versus-lymphoma immune surveillance can overcome the
      immunosuppressive PTCL microenvironment, with donor T cells targeting
      residual tumor cells.
  treatment_term:
    preferred_term: allogeneic hematopoietic stem cell transplantation
    term:
      id: NCIT:C46089
      label: Allogeneic Hematopoietic Stem Cell Transplantation
  evidence:
  - reference: PMID:20359581
    reference_title: "High-dose therapy and stem cell transplantation."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Allogeneic transplant may also have a role in relapsed PTCL, especially in the context of reduced-intensity conditioning, which has decreased nonrelapse mortality."
    explanation: >-
      This review supports an allogeneic transplant option in selected relapsed
      PTCL.
mappings:
  mondo_mappings:
  - term:
      id: MONDO:0015760
      label: T-cell non-Hodgkin lymphoma
    mapping_predicate: skos:closeMatch
    mapping_source: MONDO
    mapping_justification: >-
      MONDO does not currently provide an exact family-level peripheral T-cell
      lymphoma umbrella term. This entry therefore anchors to the closest MONDO
      T-cell non-Hodgkin lymphoma family class, while the PTCL-specific histology
      axis is carried by flat subtype facets and NCIT-grounded histopathology.
classifications:
  icdo_morphology:
    classification_value: Lymphoma
    evidence:
    - reference: PMID:36010351
      reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: "Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
      explanation: >-
        This supports classifying PTCL within lymphoma morphology.
  harrisons_chapter:
  - classification_value: ONCOLOGY_HEMATOLOGY
    evidence:
    - reference: PMID:36010351
      reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: "They predominantly affect adults and elderly people and usually exhibit an aggressive clinical course with poor prognosis."
      explanation: >-
        PTCL is an aggressive malignant neoplasm and belongs within the cancer
        chapter framework.
    - reference: PMID:36010351
      reference_title: "Pathological and Molecular Features of Nodal Peripheral T-Cell Lymphomas."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: "Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
      explanation: >-
        This supports PTCL as a hematologic malignancy.
datasets:
- accession: ega:EGAS00001000557
  title: Whole exome sequencing of peripheral T-cell lymphoma (PTCL)
  description: Somatic mutations in 3 AITL and 3 PTCL, not otherwise specified (PTCL-NOS) specimens were explored using whole-exome sequencing.
  organism:
    preferred_term: human
    term:
      id: NCBITaxon:9606
      label: Homo sapiens
  notes: 'European Genome-phenome Archive study, matched because the disease is named in the study''s own title ("Peripheral T-Cell Lymphoma"); description-level mentions were not accepted. EGA study_type: Other. Controlled access -- data require a Data Access Agreement. EGA metadata retrieved 2026-08-01.'
- accession: ega:EGAS50000001258
  title: Genetic and Microenvironmental Analysis of Peripheral T-cell Lymphoma
  description: This study enrolled 129 patients diagnosed with PTCL, comprising 94 cases of nTFHL and 35 cases of PTCL-NOS. Whole exome sequencing (WES) and RNA sequencing (RNA-seq) were performed using DNA and RNA of sufficient quality extracted from their tumor specimens. WES was conducted for all 129 cases, while RNA-seq was performed on 57 tumor samples.
  organism:
    preferred_term: human
    term:
      id: NCBITaxon:9606
      label: Homo sapiens
  data_type: WES
  notes: 'European Genome-phenome Archive study, matched because the disease is named in the study''s own title ("Peripheral T-Cell Lymphoma"); description-level mentions were not accepted. EGA study_type: Exome Sequencing. Controlled access -- data require a Data Access Agreement. EGA metadata retrieved 2026-08-01.'
📚

References & Deep Research

Deep Research

1
Peripheral T-Cell Lymphoma Deep Research Notes ▸

Peripheral T-Cell Lymphoma Deep Research Notes

Scope and modeling choice

This curation follows the cancer-entry guidance from dismech issue #1198.

  • The dismech page is treated as the mechanism-graph unit, not as one page per ontology subclass.
  • Peripheral T-cell lymphoma is modeled as a family-level nodal/systemic PTCL entry with flat subtype facets rather than separate dismech pages for each histologic entity.
  • The subtype axis is intentionally flat and histology-focused:
  • PTCL-NOS
  • TFH Angioimmunoblastic-Type
  • TFH Follicular-Type
  • TFH NOS
  • ALK-Positive ALCL
  • ALK-Negative ALCL
  • disease_term stays MONDO-first, but MONDO does not currently expose an exact family-level PTCL umbrella term. The entry therefore uses the closest MONDO family anchor, MONDO:0015760 (T-cell non-Hodgkin lymphoma), and carries oncology-specific specificity through NCIT subtype-linked histopathology and regimen/procedure terms.
  • NCIT is used preferentially where it is materially more specific for oncology:
  • disease/subtype histopathology anchors (NCIT:C3468, C4340, C7528, C80375, C139011, C37195, C37196)
  • regimen/procedure/treatment terms (NCIT:C9549, C159558, C16039, C46089)

Disease identity and subtype structure

  • PMID:36010351
  • "Peripheral T-cell lymphomas (PTCLs) are uncommon neoplasms derived from mature T cells or NK cells."
  • "According to their presentation, PTCLs can be divided into nodal, extranodal or cutaneous, and leukemic types."
  • "Nodal PTCLs include ALK-positive and ALK-negative anaplastic large cell lymphoma; nodal T-cell lymphoma with T follicular helper cell origin; and PTCL, not otherwise specified."
  • PMID:31562134
  • "Peripheral T-cell lymphoma (PTCL) is a heterogeneous group of mature T-cell malignancies; approximately one-third of cases are designated as PTCL-not otherwise specified (PTCL-NOS)."
  • PMID:36793612
  • "Three main subtypes of nodal TFH lymphomas have been described: 1) angioimmunoblastic-type, 2) follicular-type, and 3) not otherwise specified (NOS)."

Key mechanistic branches used in the YAML

1. TFH-lineage epigenetic branch

  • PMID:36793612
  • "These neoplasms feature a characteristic and similar, but not identical, mutational landscape with mutations in epigenetic modifiers (TET2, DNMT3A, IDH2), RHOA, and T-cell receptor signaling genes."
  • PMID:37841428
  • "Mutations in the epigenetic regulator TET2 are among the most frequent mutations identified in PTCL, with the highest frequency in angioimmunoblastic T cell lymphomas and other nodal T follicular helper (TFH) lymphomas."

2. RHOA/VAV1 and TCR signaling branch

  • PMID:28832024
  • "We found that binding of G17V RHOA to VAV1 augmented its adaptor function through phosphorylation of 174Tyr, resulting in acceleration of T-cell receptor (TCR) signaling."
  • "Enrichment of cytokine and chemokine-related pathways was also evident by the expression of G17V RHOA."

3. PTCL-NOS transcriptional-polarization branch

  • PMID:31562134
  • "Using gene-expression profiling (GEP), we have previously defined 2 major molecular subtypes of PTCL-NOS, PTCL-GATA3 and PTCL-TBX21, which have distinct biological differences in oncogenic pathways and prognosis."
  • PMID:30782609
  • "PTCL-GATA3 exhibited greater genomic complexity that was characterized by frequent loss or mutation of tumor suppressor genes targeting the CDKN2A /B-TP53 axis and PTEN-PI3K pathways. Co-occurring gains/amplifications of STAT3 and MYC occurred in PTCL-GATA3."
  • PMID:35639959
  • "Genomewide methylation analysis of DNMT3A-mutant vs wild-type (WT) PTCL-TBX21 cases demonstrated hypomethylation in target genes regulating interferon-γ (IFN-γ), T-cell receptor signaling, and EOMES (eomesodermin), a master transcriptional regulator of cytotoxic effector cells."

4. Immune microenvironment branch

  • PMID:38813724
  • "The PTCL TME contained a larger proportion of regulatory T cells and exhausted CD8+ T cells, with enriched expression of druggable immune checkpoints."

Clinical phenotype and pathology points used

  • PMID:20702104
  • "In the lymph node, PTCL-NOS shows paracortical or diffuse infiltrates with effacement of the normal architecture, with a broad cytological spectrum and a frequently observed inflammatory background."
  • "Patients often have B symptoms, generalized lymphadenopathy, bone marrow infiltration, and extranodal involvement, with high or high-intermediate IPI score in 50-70% of cases."
  • PMID:29302559
  • "Patients can experience night sweats, fever, lymphadenopathy, weight loss, splenomegaly, and/or skin changes."
  • "Common laboratory tests reveal that patients have anemia, thrombocytosis, lymphocytosis, eosinophilia, hypergammaglobulinemia, or increased lactate dehydrogenase."

Treatment evidence carried into the YAML

  • PMID:30914464
  • "The median PFS was 48.2 months with BV+CHP versus 20.8 months with CHOP, resulting in a hazard ratio (HR) of 0.71 (95% confidence interval [CI]: 0.54-0.93)."
  • "The trial also demonstrated improvement in overall survival (HR 0.66; 95% CI: 0.46-0.95), complete response rate (68% vs. 56%), and overall response rate (83% vs. 72%) with BV+CHP."
  • "Improvement in progression-free and overall survival over cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, which has been the standard of care for decades, is unprecedented."
  • PMID:21245435
  • "The response rate in 109 evaluable patients was 29% (32 of 109), including 12 complete responses (11%) and 20 partial responses (18%), with a median DoR of 10.1 months."
  • PMID:21355097
  • "Complete responses were observed in 8 and partial responses in 9 of 45 patients, for an overall response rate of 38% (95% confidence interval 24%-53%)."
  • "The histone deacetylase inhibitor romidepsin has single agent clinical activity associated with durable responses in patients with relapsed PTCL."
  • PMID:20359581
  • "Studies show that autologous transplant is feasible in relapsed and previously untreated patients, and efficacy is comparable to results in aggressive B-cell lymphomas."
  • "Allogeneic transplant may also have a role in relapsed PTCL, especially in the context of reduced-intensity conditioning, which has decreased nonrelapse mortality."

Outcome and epidemiology anchors

  • PMID:21138864
  • "Peripheral T-cell lymphomas (PTCL) constitute a group of heterogeneous diseases that are uncommon, representing, in Western countries, only approximately 10% of all non-Hodgkin lymphomas."
  • PMID:38532575
  • "The overall response rate to first-line therapy was 68%, while 5- and 10-year overall survival estimates were 49% and 40% respectively."
  • "Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%."

Ontology terms selected

Disease and subtype grounding

  • Disease anchor: MONDO:0015760 T-cell non-Hodgkin lymphoma
  • PTCL-NOS: MONDO:0004964
  • TFH angioimmunoblastic-type: MONDO:0004977
  • TFH follicular-type: MONDO:0958095
  • ALK-positive ALCL: MONDO:0017602
  • ALK-negative ALCL: MONDO:0017603

NCIT subtype and oncology anchors

  • PTCL family: NCIT:C3468 Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma
  • PTCL-NOS: NCIT:C4340
  • TFH lymphoma, angioimmunoblastic-type: NCIT:C7528
  • TFH lymphoma, follicular-type: NCIT:C80375
  • TFH lymphoma, NOS: NCIT:C139011
  • Systemic ALCL, ALK-positive: NCIT:C37195
  • Systemic ALCL, ALK-negative: NCIT:C37196

Core GO / CL / HP terms used

  • CL:0002038 T follicular helper cell
  • CL:0000815 regulatory T cell
  • CL:0011025 exhausted T cell
  • CL:0000794 CD8-positive, alpha-beta cytotoxic T cell
  • GO:0006325 chromatin organization
  • GO:0050852 T cell receptor signaling pathway
  • GO:0001819 positive regulation of cytokine production
  • GO:0045580 regulation of T cell differentiation
  • GO:0007259 cell surface receptor signaling pathway via JAK-STAT
  • GO:0050777 negative regulation of immune response
  • HP:0002716 Lymphadenopathy
  • HP:0001945 Fever
  • HP:0030166 Night sweats
  • HP:0001824 Weight loss
  • HP:0001744 Splenomegaly
  • HP:0001903 Anemia