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)

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)."

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."

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."

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."

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."

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."

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."

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%."