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0
Mappings
0
Definitions
0
Inheritance
6
Pathophysiology
1
Histopathology
7
Phenotypes
6
Pathograph
4
Genes
6
Treatments
3
Subtypes
0
Differentials
0
Datasets
0
Trials
0
Models
14
References
1
Deep Research
🏷

Classifications

Harrison's Chapter
cancer hematologic malignancy
ICD-O Morphology
Lymphoma

Subtypes

3
Classic Follicular Lymphoma
Conventional nodal FL with a follicular growth pattern and frequent t(14;18)(q32;q21)/IGH::BCL2 translocation. Usually composed of centrocytes with variable centroblasts and follows an indolent relapsing course.
t(14;18)-Negative Follicular Lymphoma
Minority subset lacking IGH::BCL2 rearrangement. These cases can show alternative genetic drivers and may require more careful clinicopathologic and molecular correlation for diagnosis.
Transformed Follicular Lymphoma
Histologic progression from indolent FL to aggressive large B-cell lymphoma, typically accompanied by increased proliferation, loss of follicular architecture, and worse prognosis.

Pathophysiology

6
BCL2 Overexpression
In most cases, the hallmark t(14;18)(q32;q21)/IGH::BCL2 rearrangement places BCL2 under immunoglobulin enhancer control, driving constitutive BCL2 overexpression in germinal center B cells.
germinal center B cell link
Show evidence (1 reference)
PMID:39490765 SUPPORT
"Approximately 85% of FL cases harbor the t(14;18)(q32;q21)/IGH::BCL2 which leads to the overexpression of BCL2."
Abstract identifies the hallmark IGH::BCL2 rearrangement and resulting BCL2 overexpression in classic FL.
Apoptosis Resistance
Constitutive BCL2 expression blocks the mitochondrial apoptosis program, allowing germinal center B cells that would normally be deleted during the germinal center reaction to survive as lymphoma precursors.
apoptotic process link ↓ DECREASED
Show evidence (1 reference)
PMID:38536645 SUPPORT
"BCL2 overexpression by t(14;18) confers a survival advantage to B cells during the germinal center reaction"
Review abstract states that t(14;18)-driven BCL2 expression provides a survival advantage during the germinal center reaction.
Epigenetic Deregulation of Germinal Center Program
FL commonly carries recurrent mutations in chromatin-modifying genes including KMT2D, CREBBP, and EZH2. These lesions distort transcriptional programs required for normal germinal center exit and differentiation, reinforcing the malignant state.
germinal center B cell link
germinal center B cell differentiation link ↕ DYSREGULATED
Show evidence (2 references)
PMID:39490765 SUPPORT
"These neoplasms often exhibit hallmark epigenetic deregulation due to recurrent mutations in genes such as KMT2D, CREBBP, and EZH2"
Abstract directly links FL to recurrent mutations in major chromatin-modifying genes.
PMID:38536645 SUPPORT
"abnormalities in epigenetic modifier genes lead to desynchronization of gene expression changes in germinal center B cells."
Abstract supports disruption of the normal germinal center transcriptional program by epigenetic lesions.
Microenvironmental Supportive Synapse
FL cells remain highly dependent on the lymph node microenvironment. Reciprocal signaling with follicular helper T cells, stromal cells, and other immune cells provides survival, adhesion, and immune-evasive signals that preserve tumor fitness.
follicular helper T cell link fibroblast link
B cell activation link ↑ INCREASED
Show evidence (1 reference)
PMID:33028033 SUPPORT
"Follicular lymphoma (FL), the most frequent indolent non-Hodgkin's B cell lymphoma, is considered as a prototypical centrocyte-derived lymphoma, dependent on a specific microenvironment mimicking the normal germinal center (GC)."
Abstract states that FL biology depends on a supportive microenvironment resembling the normal germinal center.
Chronic B-Cell Receptor Signaling
FL immunoglobulins often acquire N-linked glycosylation motifs that support chronic B-cell receptor signaling. This persistent signaling promotes dark-zone polarization and ongoing clonal evolution.
B cell receptor signaling pathway link ↑ INCREASED
Show evidence (1 reference)
PMID:38536645 SUPPORT
"FL cells frequently carry N-linked glycosylation motifs within the immunoglobulin gene, leading to chronic activation of the B-cell receptor (BCR). Recent evidence suggests that this chronic BCR signaling drives FL polarization toward a dark-zone phenotype and promotes clonal evolution."
Review abstract directly links chronic BCR signaling to clonal evolution in FL.
Clonal Persistence and Evolution
The combination of anti-apoptotic signaling, epigenetic deregulation, and microenvironmental support, and chronic BCR signaling allows FL clones to survive for years, relapse after therapy, and occasionally transform into aggressive large B-cell lymphoma.
germinal center B cell link

Histopathology

1
Follicular Lymphoma VERY_FREQUENT
Follicular lymphoma is a germinal center B-cell neoplasm with at least a partial follicular growth pattern.
Show evidence (1 reference)
PMID:39490765 SUPPORT
"Follicular lymphoma (FL) is a neoplasm that originates from germinal center B cells and typically forms at least a partial follicular pattern."
Abstract defines FL as a germinal center B-cell neoplasm with follicular architecture.

Pathograph

Use the checkboxes to hide or show graph categories. Hover nodes for evidence and cross-linked metadata.
Pathograph: causal mechanism network for Follicular 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

7
Blood 1
Anemia OCCASIONAL Anemia (HP:0001903)
Cardiovascular 2
Generalized Lymphadenopathy VERY_FREQUENT Generalized lymphadenopathy (HP:0008940)
Splenomegaly FREQUENT Splenomegaly (HP:0001744)
Constitutional 2
Fatigue FREQUENT Fatigue (HP:0012378)
Night Sweats OCCASIONAL Night sweats (HP:0030166)
Growth 1
Weight Loss OCCASIONAL Weight loss (HP:0001824)
Other 1
Bone Marrow Involvement FREQUENT Abnormal bone marrow cell morphology (HP:0005561)
Show evidence (1 reference)
DOI:10.1002/ajh.26737 SUPPORT Human Clinical
"FL is characterized by diffuse lymphadenopathy, bone marrow involvement, and splenomegaly."
Review identifies bone marrow involvement as a characteristic clinical feature of FL.
🧬

Genetic Associations

4
BCL2/IGH Translocation (Defining Genetic Lesion)
KMT2D (Founding Mutation)
CREBBP (Founding Mutation)
EZH2 (Recurrent Somatic Mutation)
💊

Treatments

6
Anti-CD20-Based Immunochemotherapy
Action: chemotherapy MAXO:0000647
Agent: rituximab bendamustine
Standard therapy for symptomatic advanced FL combines an anti-CD20 antibody such as rituximab or obinutuzumab with chemotherapy backbones such as bendamustine, CHOP, or CVP, depending on patient fitness and disease burden.
Lenalidomide Plus Anti-CD20 Therapy
Action: pharmacotherapy MAXO:0000058
Agent: lenalidomide rituximab
The chemo-free lenalidomide plus rituximab regimen is an established option for selected patients in frontline or relapsed settings.
Tazemetostat
Action: pharmacotherapy MAXO:0000058
Agent: tazemetostat
Oral EZH2 inhibition with tazemetostat is used in relapsed or refractory FL, especially in tumors with EZH2 mutation, and offers a targeted option with relatively favorable tolerability.
Mosunetuzumab
Action: immunotherapy Ontology label: immunotherapy procedure MAXO:0001002
Agent: mosunetuzumab
CD20xCD3 bispecific antibody immunotherapy for relapsed or refractory FL after at least two prior systemic therapy lines, supported by the GO29781 phase I/II program.
Show evidence (1 reference)
DOI:10.3324/haematol.2023.283737 SUPPORT Human Clinical
"A recent pivotal phase I/II clinical trial (GO29781) demonstrated that mosunetuzumab induced an overall response rate of 80%, complete response rate of 60%, and a median progression-free survival of 17.9 months in patients with relapsed/refractory (r/r) follicular lymphoma (FL) following at..."
Phase I/II clinical trial data support mosunetuzumab as an active therapy for relapsed or refractory FL after multiple prior lines.
Axicabtagene Ciloleucel
Action: immunotherapy Ontology label: immunotherapy procedure MAXO:0001002
Agent: axicabtagene ciloleucel
Autologous anti-CD19 CAR-T cell therapy used for relapsed or refractory follicular lymphoma after prior anti-CD20 antibody and alkylator-based therapy, supported by the ZUMA-5 phase II trial.
Show evidence (1 reference)
PMID:34895487 SUPPORT Human Clinical
"Axicabtagene ciloleucel showed high rates of durable responses"
ZUMA-5 provides phase II clinical evidence for axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma including FL.
Tisagenlecleucel
Action: immunotherapy Ontology label: immunotherapy procedure MAXO:0001002
Agent: tisagenlecleucel
Autologous anti-CD19 CAR-T cell therapy for adults with relapsed or refractory follicular lymphoma after multiple prior treatment lines, supported by the ELARA phase II trial.
Show evidence (1 reference)
PMID:34921238 SUPPORT Human Clinical
"Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell"
ELARA provides phase II clinical evidence for tisagenlecleucel in extensively pretreated relapsed or refractory FL.
🔬

Biochemical Markers

2
Germinal Center Immunophenotype
Elevated Lactate Dehydrogenase (LDH)
{ }

Source YAML

click to show
name: Follicular Lymphoma
creation_date: '2026-04-12T05:13:04Z'
updated_date: '2026-05-09T00:41:13Z'
description: >-
  Follicular lymphoma (FL) is an indolent germinal center B-cell non-Hodgkin
  lymphoma that usually arises from precursor cells harboring the
  t(14;18)(q32;q21)/IGH::BCL2 translocation. The disease is characterized by
  follicular growth, recurrent mutations in chromatin-modifying genes such as
  KMT2D, CREBBP, and EZH2, and sustained dependence on a supportive immune and
  stromal microenvironment. FL typically follows a relapsing course with long
  survival but retains a persistent risk of histologic transformation to
  aggressive large B-cell lymphoma.
categories:
- Hematologic Malignancy
- B-cell Neoplasm
- Non-Hodgkin Lymphoma
parents:
- B-cell non-Hodgkin lymphoma
has_subtypes:
- name: Classic Follicular Lymphoma
  description: >-
    Conventional nodal FL with a follicular growth pattern and frequent
    t(14;18)(q32;q21)/IGH::BCL2 translocation. Usually composed of centrocytes
    with variable centroblasts and follows an indolent relapsing course.
- name: t(14;18)-Negative Follicular Lymphoma
  description: >-
    Minority subset lacking IGH::BCL2 rearrangement. These cases can show
    alternative genetic drivers and may require more careful clinicopathologic
    and molecular correlation for diagnosis.
- name: Transformed Follicular Lymphoma
  description: >-
    Histologic progression from indolent FL to aggressive large B-cell lymphoma,
    typically accompanied by increased proliferation, loss of follicular
    architecture, and worse prognosis.
pathophysiology:
- name: BCL2 Overexpression
  description: >-
    In most cases, the hallmark t(14;18)(q32;q21)/IGH::BCL2 rearrangement
    places BCL2 under immunoglobulin enhancer control, driving constitutive
    BCL2 overexpression in germinal center B cells.
  evidence:
  - reference: PMID:39490765
    reference_title: 'Bridging clinicopathologic features and genetics in follicular lymphoma: Towards enhanced diagnostic accuracy and subtype differentiation.'
    supports: SUPPORT
    snippet: "Approximately 85% of FL cases harbor the t(14;18)(q32;q21)/IGH::BCL2 which leads to the overexpression of BCL2."
    explanation: Abstract identifies the hallmark IGH::BCL2 rearrangement and resulting BCL2 overexpression in classic FL.
  cell_types:
  - preferred_term: germinal center B cell
    term:
      id: CL:0000844
      label: germinal center B cell
  downstream:
  - target: Apoptosis Resistance
    description: BCL2 overexpression prevents deletion of germinal center B cells
  - target: Clonal Persistence and Evolution
    description: Oncogenic BCL2 expression helps establish long-lived precursor clones
- name: Apoptosis Resistance
  description: >-
    Constitutive BCL2 expression blocks the mitochondrial apoptosis program,
    allowing germinal center B cells that would normally be deleted during the
    germinal center reaction to survive as lymphoma precursors.
  evidence:
  - reference: PMID:38536645
    reference_title: Recent advances in understanding the biology of follicular lymphoma.
    supports: SUPPORT
    snippet: "BCL2 overexpression by t(14;18) confers a survival advantage to B cells during the germinal center reaction"
    explanation: Review abstract states that t(14;18)-driven BCL2 expression provides a survival advantage during the germinal center reaction.
  biological_processes:
  - preferred_term: apoptotic process
    modifier: DECREASED
    term:
      id: GO:0006915
      label: apoptotic process
  downstream:
  - target: Clonal Persistence and Evolution
    description: Anti-apoptotic signaling allows precursor and tumor B cells to persist long enough to accumulate additional lesions
- name: Epigenetic Deregulation of Germinal Center Program
  description: >-
    FL commonly carries recurrent mutations in chromatin-modifying genes
    including KMT2D, CREBBP, and EZH2. These lesions distort transcriptional
    programs required for normal germinal center exit and differentiation,
    reinforcing the malignant state.
  evidence:
  - reference: PMID:39490765
    reference_title: 'Bridging clinicopathologic features and genetics in follicular lymphoma: Towards enhanced diagnostic accuracy and subtype differentiation.'
    supports: SUPPORT
    snippet: "These neoplasms often exhibit hallmark epigenetic deregulation due to recurrent mutations in genes such as KMT2D, CREBBP, and EZH2"
    explanation: Abstract directly links FL to recurrent mutations in major chromatin-modifying genes.
  - reference: PMID:38536645
    reference_title: Recent advances in understanding the biology of follicular lymphoma.
    supports: SUPPORT
    snippet: "abnormalities in epigenetic modifier genes lead to desynchronization of gene expression changes in germinal center B cells."
    explanation: Abstract supports disruption of the normal germinal center transcriptional program by epigenetic lesions.
  cell_types:
  - preferred_term: germinal center B cell
    term:
      id: CL:0000844
      label: germinal center B cell
  biological_processes:
  - preferred_term: germinal center B cell differentiation
    modifier: DYSREGULATED
    term:
      id: GO:0002314
      label: germinal center B cell differentiation
  downstream:
  - target: Clonal Persistence and Evolution
    description: Distorted chromatin programs stabilize malignant identity and support ongoing clonal diversification
- name: Microenvironmental Supportive Synapse
  description: >-
    FL cells remain highly dependent on the lymph node microenvironment.
    Reciprocal signaling with follicular helper T cells, stromal cells, and
    other immune cells provides survival, adhesion, and immune-evasive signals
    that preserve tumor fitness.
  evidence:
  - reference: PMID:33028033
    reference_title: 'Integrative Analysis of Cell Crosstalk within Follicular Lymphoma Cell Niche: Towards a Definition of the FL Supportive Synapse.'
    supports: SUPPORT
    snippet: "Follicular lymphoma (FL), the most frequent indolent non-Hodgkin's B cell lymphoma, is considered as a prototypical centrocyte-derived lymphoma, dependent on a specific microenvironment mimicking the normal germinal center (GC)."
    explanation: Abstract states that FL biology depends on a supportive microenvironment resembling the normal germinal center.
  cell_types:
  - preferred_term: follicular helper T cell
    term:
      id: CL:0002038
      label: T follicular helper cell
  - preferred_term: fibroblast
    term:
      id: CL:0000057
      label: fibroblast
  biological_processes:
  - preferred_term: B cell activation
    modifier: INCREASED
    term:
      id: GO:0042113
      label: B cell activation
  downstream:
  - target: Clonal Persistence and Evolution
    description: Sustained trophic signaling from the niche promotes relapse-prone survival and immune escape
- name: Chronic B-Cell Receptor Signaling
  description: >-
    FL immunoglobulins often acquire N-linked glycosylation motifs that support
    chronic B-cell receptor signaling. This persistent signaling promotes
    dark-zone polarization and ongoing clonal evolution.
  evidence:
  - reference: PMID:38536645
    reference_title: Recent advances in understanding the biology of follicular lymphoma.
    supports: SUPPORT
    snippet: "FL cells frequently carry N-linked glycosylation motifs within the immunoglobulin gene, leading to chronic activation of the B-cell receptor (BCR). Recent evidence suggests that this chronic BCR signaling drives FL polarization toward a dark-zone phenotype and promotes clonal evolution."
    explanation: Review abstract directly links chronic BCR signaling to clonal evolution in FL.
  biological_processes:
  - preferred_term: B cell receptor signaling pathway
    modifier: INCREASED
    term:
      id: GO:0050853
      label: B cell receptor signaling pathway
  downstream:
  - target: Clonal Persistence and Evolution
    description: Persistent BCR signaling supports ongoing adaptation and outgrowth of FL clones
- name: Clonal Persistence and Evolution
  description: >-
    The combination of anti-apoptotic signaling, epigenetic deregulation, and
    microenvironmental support, and chronic BCR signaling allows FL clones to
    survive for years, relapse after therapy, and occasionally transform into
    aggressive large B-cell lymphoma.
  cell_types:
  - preferred_term: germinal center B cell
    term:
      id: CL:0000844
      label: germinal center B cell
histopathology:
- name: Follicular Lymphoma
  finding_term:
    preferred_term: Follicular Lymphoma
    term:
      id: NCIT:C3209
      label: Follicular Lymphoma
  frequency: VERY_FREQUENT
  description: Follicular lymphoma is a germinal center B-cell neoplasm with at least a partial follicular growth pattern.
  evidence:
  - reference: PMID:39490765
    reference_title: 'Bridging clinicopathologic features and genetics in follicular lymphoma: Towards enhanced diagnostic accuracy and subtype differentiation.'
    supports: SUPPORT
    snippet: "Follicular lymphoma (FL) is a neoplasm that originates from germinal center B cells and typically forms at least a partial follicular pattern."
    explanation: Abstract defines FL as a germinal center B-cell neoplasm with follicular architecture.
phenotypes:
- category: Lymphatic
  name: Generalized Lymphadenopathy
  frequency: VERY_FREQUENT
  description: >-
    Painless lymph node enlargement is the most common presentation, often
    involving multiple nodal stations at diagnosis.
  phenotype_term:
    preferred_term: Generalized lymphadenopathy
    term:
      id: HP:0008940
      label: Generalized lymphadenopathy
- category: Abdominal
  name: Splenomegaly
  frequency: FREQUENT
  description: >-
    Splenic enlargement is common in disseminated disease and may accompany bone
    marrow involvement.
  phenotype_term:
    preferred_term: Splenomegaly
    term:
      id: HP:0001744
      label: Splenomegaly
- category: Hematologic
  name: Bone Marrow Involvement
  frequency: FREQUENT
  description: >-
    Bone marrow involvement is a common manifestation of disseminated follicular
    lymphoma and can contribute to cytopenias when marrow hematopoiesis is
    displaced.
  phenotype_term:
    preferred_term: Bone marrow involvement
    term:
      id: HP:0005561
      label: Abnormal bone marrow cell morphology
  evidence:
  - reference: DOI:10.1002/ajh.26737
    reference_title: 'Follicular lymphoma: 2023 update on diagnosis and management'
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "FL is characterized by diffuse lymphadenopathy, bone marrow involvement, and splenomegaly."
    explanation: Review identifies bone marrow involvement as a characteristic clinical feature of FL.
- category: Constitutional
  name: Fatigue
  frequency: FREQUENT
  description: >-
    Fatigue reflects chronic tumor burden, cytokine effects, and in some
    patients accompanying cytopenias.
  phenotype_term:
    preferred_term: Fatigue
    term:
      id: HP:0012378
      label: Fatigue
- category: Constitutional
  name: Night Sweats
  frequency: OCCASIONAL
  description: >-
    Drenching night sweats are part of the B-symptom complex and usually reflect
    more active or bulky disease.
  phenotype_term:
    preferred_term: Night sweats
    term:
      id: HP:0030166
      label: Night sweats
- category: Constitutional
  name: Weight Loss
  frequency: OCCASIONAL
  description: >-
    Unintentional weight loss is less common in indolent FL than in aggressive
    lymphomas but may accompany symptomatic advanced disease or transformation.
  phenotype_term:
    preferred_term: Weight loss
    term:
      id: HP:0001824
      label: Weight loss
- category: Hematologic
  name: Anemia
  frequency: OCCASIONAL
  description: >-
    Anemia can result from marrow infiltration, chronic inflammation, or
    treatment-related myelosuppression.
  phenotype_term:
    preferred_term: Anemia
    term:
      id: HP:0001903
      label: Anemia
biochemical:
- name: Germinal Center Immunophenotype
  notes: >-
    Neoplastic cells typically express pan-B-cell markers together with germinal
    center markers such as CD10 and BCL6, with aberrant BCL2 expression in most
    classic t(14;18)-positive cases.
- name: Elevated Lactate Dehydrogenase (LDH)
  notes: >-
    LDH may increase with higher tumor burden or histologic transformation and
    remains a common prognostic laboratory marker in FL.
genetic:
- name: BCL2/IGH Translocation
  gene_term:
    preferred_term: BCL2
    term:
      id: hgnc:990
      label: BCL2
  association: Defining Genetic Lesion
  notes: >-
    The hallmark t(14;18)(q32;q21)/IGH::BCL2 rearrangement is present in most
    classic FL cases and promotes lymphoma cell survival through BCL2
    overexpression.
- name: KMT2D
  gene_term:
    preferred_term: KMT2D
    term:
      id: hgnc:7133
      label: KMT2D
  association: Founding Mutation
  notes: >-
    KMT2D loss-of-function mutations are among the most frequent early lesions
    in FL and contribute to aberrant enhancer regulation and germinal center
    reprogramming.
- name: CREBBP
  gene_term:
    preferred_term: CREBBP
    term:
      id: hgnc:2348
      label: CREBBP
  association: Founding Mutation
  notes: >-
    CREBBP mutations impair acetylation-dependent transcriptional control,
    affecting antigen presentation and germinal center exit programs.
- name: EZH2
  gene_term:
    preferred_term: EZH2
    term:
      id: hgnc:3527
      label: EZH2
  association: Recurrent Somatic Mutation
  notes: >-
    Gain-of-function EZH2 mutations alter histone methylation, reinforce the
    germinal center phenotype, and provide a targetable vulnerability in a
    subset of relapsed FL.
treatments:
- name: Anti-CD20-Based Immunochemotherapy
  description: >-
    Standard therapy for symptomatic advanced FL combines an anti-CD20 antibody
    such as rituximab or obinutuzumab with chemotherapy backbones such as
    bendamustine, CHOP, or CVP, depending on patient fitness and disease burden.
  treatment_term:
    preferred_term: chemotherapy
    term:
      id: MAXO:0000647
      label: chemotherapy
    therapeutic_agent:
    - preferred_term: rituximab
      term:
        id: NCIT:C1702
        label: Rituximab
    - preferred_term: bendamustine
      term:
        id: CHEBI:135515
        label: bendamustine
- name: Lenalidomide Plus Anti-CD20 Therapy
  description: >-
    The chemo-free lenalidomide plus rituximab regimen is an established option
    for selected patients in frontline or relapsed settings.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    therapeutic_agent:
    - preferred_term: lenalidomide
      term:
        id: CHEBI:63791
        label: lenalidomide
    - preferred_term: rituximab
      term:
        id: NCIT:C1702
        label: Rituximab
- name: Tazemetostat
  description: >-
    Oral EZH2 inhibition with tazemetostat is used in relapsed or refractory FL,
    especially in tumors with EZH2 mutation, and offers a targeted option with
    relatively favorable tolerability.
  treatment_term:
    preferred_term: pharmacotherapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    therapeutic_agent:
    - preferred_term: tazemetostat
      term:
        id: CHEBI:231598
        label: tazemetostat
- name: Mosunetuzumab
  description: >-
    CD20xCD3 bispecific antibody immunotherapy for relapsed or refractory FL
    after at least two prior systemic therapy lines, supported by the GO29781
    phase I/II program.
  treatment_term:
    preferred_term: immunotherapy
    term:
      id: MAXO:0001002
      label: immunotherapy procedure
    therapeutic_agent:
    - preferred_term: mosunetuzumab
      term:
        id: NCIT:C129691
        label: Mosunetuzumab
  evidence:
  - reference: DOI:10.3324/haematol.2023.283737
    reference_title: Matching-adjusted indirect comparison from the Lymphoma Epidemiology of Outcomes Consortium for Real World Evidence (LEO CReWE) study to a clinical trial of mosunetuzumab in relapsed or refractory follicular lymphoma
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "A recent pivotal phase I/II clinical trial (GO29781) demonstrated that mosunetuzumab induced an overall response rate of 80%, complete response rate of 60%, and a median progression-free survival of 17.9 months in patients with relapsed/refractory (r/r) follicular lymphoma (FL) following at least two prior lines of systemic therapy, including alkylator and anti-CD20 antibody-based therapy."
    explanation: Phase I/II clinical trial data support mosunetuzumab as an active therapy for relapsed or refractory FL after multiple prior lines.
- name: Axicabtagene Ciloleucel
  description: >-
    Autologous anti-CD19 CAR-T cell therapy used for relapsed or refractory
    follicular lymphoma after prior anti-CD20 antibody and alkylator-based
    therapy, supported by the ZUMA-5 phase II trial.
  treatment_term:
    preferred_term: immunotherapy
    term:
      id: MAXO:0001002
      label: immunotherapy procedure
    therapeutic_agent:
    - preferred_term: axicabtagene ciloleucel
      term:
        id: NCIT:C120309
        label: Axicabtagene Ciloleucel
  evidence:
  - reference: PMID:34895487
    reference_title: "Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Axicabtagene ciloleucel showed high rates of durable responses"
    explanation: ZUMA-5 provides phase II clinical evidence for axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma including FL.
- name: Tisagenlecleucel
  description: >-
    Autologous anti-CD19 CAR-T cell therapy for adults with relapsed or
    refractory follicular lymphoma after multiple prior treatment lines,
    supported by the ELARA phase II trial.
  treatment_term:
    preferred_term: immunotherapy
    term:
      id: MAXO:0001002
      label: immunotherapy procedure
    therapeutic_agent:
    - preferred_term: tisagenlecleucel
      term:
        id: NCIT:C102758
        label: Tisagenlecleucel
  evidence:
  - reference: PMID:34921238
    reference_title: "Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial."
    supports: SUPPORT
    evidence_source: HUMAN_CLINICAL
    snippet: "Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell"
    explanation: ELARA provides phase II clinical evidence for tisagenlecleucel in extensively pretreated relapsed or refractory FL.
disease_term:
  preferred_term: follicular lymphoma
  term:
    id: MONDO:0018906
    label: follicular lymphoma
classifications:
  icdo_morphology:
    classification_value: Lymphoma
  harrisons_chapter:
  - classification_value: cancer
  - classification_value: hematologic malignancy
references:
- reference: DOI:10.1002/ajh.26737
  title: 'Follicular lymphoma: 2023 update on diagnosis and management'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells.
    supporting_text: Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells.
    evidence:
    - reference: DOI:10.1002/ajh.26737
      reference_title: 'Follicular lymphoma: 2023 update on diagnosis and management'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.1002/hon.3138
  title: Update on follicular lymphoma
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma.
    supporting_text: The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma.
    evidence:
    - reference: DOI:10.1002/hon.3138
      reference_title: Update on follicular lymphoma
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: PMID:34895487
  title: "Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial."
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Axicabtagene ciloleucel showed high rates of durable responses.
    supporting_text: Axicabtagene ciloleucel showed high rates of durable responses.
    evidence:
    - reference: PMID:34895487
      reference_title: "Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Axicabtagene ciloleucel showed high rates of durable responses
      explanation: Phase II ZUMA-5 clinical trial evidence supports axicabtagene ciloleucel for relapsed or refractory indolent non-Hodgkin lymphoma including FL.
- reference: PMID:34921238
  title: "Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial."
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell therapy.
    supporting_text: Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell therapy.
    evidence:
    - reference: PMID:34921238
      reference_title: "Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial."
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell
      explanation: Phase II ELARA clinical trial evidence supports tisagenlecleucel for extensively pretreated relapsed or refractory FL.
- reference: DOI:10.1007/s00428-022-03432-2
  title: 'Follicular lymphoma and marginal zone lymphoma: how many diseases?'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features.
    supporting_text: Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features.
    evidence:
    - reference: DOI:10.1007/s00428-022-03432-2
      reference_title: 'Follicular lymphoma and marginal zone lymphoma: how many diseases?'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.1007/s12185-024-03834-9
  title: 'Tazemetostat for relapsed/refractory B-cell non-Hodgkin lymphoma with EZH2 mutation in Japan: 3-year follow-up for a phase II study'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: 'Tazemetostat for relapsed/refractory B-cell non-Hodgkin lymphoma with EZH2 mutation in Japan: 3-year follow-up for a phase II study'
    supporting_text: Previously, we reported the efficacy and safety of tazemetostat in Japanese patients with relapsed/refractory follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) harboring the EZH2 mutation in a multicenter, open-label, phase II study.
    evidence:
    - reference: DOI:10.1007/s12185-024-03834-9
      reference_title: 'Tazemetostat for relapsed/refractory B-cell non-Hodgkin lymphoma with EZH2 mutation in Japan: 3-year follow-up for a phase II study'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Previously, we reported the efficacy and safety of tazemetostat in Japanese patients with relapsed/refractory follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) harboring the EZH2 mutation in a multicenter, open-label, phase II study.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.1007/s12325-024-02882-1
  title: 'Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: 'Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States'
    supporting_text: 'Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States'
- reference: DOI:10.1186/s40164-024-00551-1
  title: Current and future therapies for follicular lymphoma
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival.
    supporting_text: Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival.
    evidence:
    - reference: DOI:10.1186/s40164-024-00551-1
      reference_title: Current and future therapies for follicular lymphoma
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.1186/s40364-023-00525-1
  title: 'Survival of patients with transformed follicular lymphoma in the United States: a multiple cohort study'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking.
    supporting_text: Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking.
    evidence:
    - reference: DOI:10.1186/s40364-023-00525-1
      reference_title: 'Survival of patients with transformed follicular lymphoma in the United States: a multiple cohort study'
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.3324/haematol.2023.283737
  title: Matching-adjusted indirect comparison from the Lymphoma Epidemiology of Outcomes Consortium for Real World Evidence (LEO CReWE) study to a clinical trial of mosunetuzumab in relapsed or refractory follicular lymphoma
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells.
    supporting_text: Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells.
    evidence:
    - reference: DOI:10.3324/haematol.2023.283737
      reference_title: Matching-adjusted indirect comparison from the Lymphoma Epidemiology of Outcomes Consortium for Real World Evidence (LEO CReWE) study to a clinical trial of mosunetuzumab in relapsed or refractory follicular lymphoma
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.3390/cancers15030785
  title: Follicular Lymphoma in the 5th Edition of the WHO-Classification of Haematolymphoid Neoplasms—Updated Classification and New Biological Data
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision.
    supporting_text: The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision.
    evidence:
    - reference: DOI:10.3390/cancers15030785
      reference_title: Follicular Lymphoma in the 5th Edition of the WHO-Classification of Haematolymphoid Neoplasms—Updated Classification and New Biological Data
      supports: SUPPORT
      evidence_source: OTHER
      snippet: The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.3390/cancers15174403
  title: Burden of Illness in Follicular Lymphoma with Multiple Lines of Treatment, Italian RWE Analysis
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs.
    supporting_text: This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs.
    evidence:
    - reference: DOI:10.3390/cancers15174403
      reference_title: Burden of Illness in Follicular Lymphoma with Multiple Lines of Treatment, Italian RWE Analysis
      supports: SUPPORT
      evidence_source: HUMAN_CLINICAL
      snippet: This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.3390/hemato4010003
  title: 'Classification of B-Cell Lymphomas and Immunodeficiency-Related Lymphoproliferations: What’s New?'
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: 'Classification of B-Cell Lymphomas and Immunodeficiency-Related Lymphoproliferations: What’s New?'
    supporting_text: New insights from genomic studies have had an impact on the definition and the diagnosis of several lymphoid tumors including follicular B-cell lymphomas, aggressive diffuse large B-cell lymphomas, and lymphoproliferations associated with acquired and posttransplant immunodeficiencies.
    evidence:
    - reference: DOI:10.3390/hemato4010003
      reference_title: 'Classification of B-Cell Lymphomas and Immunodeficiency-Related Lymphoproliferations: What’s New?'
      supports: SUPPORT
      evidence_source: OTHER
      snippet: New insights from genomic studies have had an impact on the definition and the diagnosis of several lymphoid tumors including follicular B-cell lymphomas, aggressive diffuse large B-cell lymphomas, and lymphoproliferations associated with acquired and posttransplant immunodeficiencies.
      explanation: Deep research cited this publication as relevant literature for Follicular Lymphoma.
- reference: DOI:10.7150/ijbs.80401
  title: Advances in the multi-omics landscape of follicular lymphoma
  found_in:
  - Follicular_Lymphoma-deep-research-falcon.md
  findings:
  - statement: Advances in the multi-omics landscape of follicular lymphoma
    supporting_text: Advances in the multi-omics landscape of follicular lymphoma
📚

References & Deep Research

References

14
Follicular lymphoma: 2023 update on diagnosis and management
1 finding
Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells.
"Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells."
Show evidence (1 reference)
DOI:10.1002/ajh.26737 SUPPORT Human Clinical
"Disease OverviewFollicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Update on follicular lymphoma
1 finding
The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma.
"The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma."
Show evidence (1 reference)
DOI:10.1002/hon.3138 SUPPORT Human Clinical
"The past two decades have seen remarkable progress in both biological understanding and optimizing treatment of follicular lymphoma."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Axicabtagene ciloleucel in relapsed or refractory indolent non-Hodgkin lymphoma (ZUMA-5): a single-arm, multicentre, phase 2 trial.
1 finding
Axicabtagene ciloleucel showed high rates of durable responses.
"Axicabtagene ciloleucel showed high rates of durable responses."
Show evidence (1 reference)
PMID:34895487 SUPPORT Human Clinical
"Axicabtagene ciloleucel showed high rates of durable responses"
Phase II ZUMA-5 clinical trial evidence supports axicabtagene ciloleucel for relapsed or refractory indolent non-Hodgkin lymphoma including FL.
Tisagenlecleucel in adult relapsed or refractory follicular lymphoma: the phase 2 ELARA trial.
1 finding
Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell therapy.
"Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell therapy."
Show evidence (1 reference)
PMID:34921238 SUPPORT Human Clinical
"Tisagenlecleucel is an autologous anti-CD19 chimeric antigen receptor-T cell"
Phase II ELARA clinical trial evidence supports tisagenlecleucel for extensively pretreated relapsed or refractory FL.
Follicular lymphoma and marginal zone lymphoma: how many diseases?
1 finding
Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features.
"Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features."
Show evidence (1 reference)
"Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are indolent mature B-cell neoplasms with variable clinical presentation and distinct histopathologic features."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Tazemetostat for relapsed/refractory B-cell non-Hodgkin lymphoma with EZH2 mutation in Japan: 3-year follow-up for a phase II study
1 finding
Tazemetostat for relapsed/refractory B-cell non-Hodgkin lymphoma with EZH2 mutation in Japan: 3-year follow-up for a phase II study
"Previously, we reported the efficacy and safety of tazemetostat in Japanese patients with relapsed/refractory follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) harboring the EZH2 mutation in a multicenter, open-label, phase II study."
Show evidence (1 reference)
DOI:10.1007/s12185-024-03834-9 SUPPORT Human Clinical
"Previously, we reported the efficacy and safety of tazemetostat in Japanese patients with relapsed/refractory follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) harboring the EZH2 mutation in a multicenter, open-label, phase II study."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States
1 finding
Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States
"Quality of Life Evaluation in Patients with Follicular Cell Lymphoma: A Real-World Study in Europe and the United States"
Current and future therapies for follicular lymphoma
1 finding
Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival.
"Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival."
Show evidence (1 reference)
DOI:10.1186/s40164-024-00551-1 SUPPORT Human Clinical
"Follicular lymphoma (FL) is an indolent, germinal center B cell–derived lymphoid neoplasm, for which recent advances in treatment have substantially improved patient survival."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Survival of patients with transformed follicular lymphoma in the United States: a multiple cohort study
1 finding
Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking.
"Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking."
Show evidence (1 reference)
DOI:10.1186/s40364-023-00525-1 SUPPORT Human Clinical
"Population-based data comparing the outcomes of patients with transformed follicular lymphoma (t-FL) and de novo diffuse large B-cell lymphoma (DLBCL) are lacking."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Matching-adjusted indirect comparison from the Lymphoma Epidemiology of Outcomes Consortium for Real World Evidence (LEO CReWE) study to a clinical trial of mosunetuzumab in relapsed or refractory follicular lymphoma
1 finding
Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells.
"Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells."
Show evidence (1 reference)
DOI:10.3324/haematol.2023.283737 SUPPORT Human Clinical
"Mosunetuzumab is a novel bispecific antibody targeting epitopes on CD3 on T cells and CD20 on B cells with the goal of inducing T-cell mediated elimination of malignant B cells."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Follicular Lymphoma in the 5th Edition of the WHO-Classification of Haematolymphoid Neoplasms—Updated Classification and New Biological Data
1 finding
The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision.
"The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision."
Show evidence (1 reference)
"The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Burden of Illness in Follicular Lymphoma with Multiple Lines of Treatment, Italian RWE Analysis
1 finding
This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs.
"This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs."
Show evidence (1 reference)
DOI:10.3390/cancers15174403 SUPPORT Human Clinical
"This real-world analysis investigated patients with follicular lymphoma in Italy receiving three or more treatment lines (≥3L), focusing on therapeutic pathways with their rebounds on healthcare resource consumptions and costs."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Classification of B-Cell Lymphomas and Immunodeficiency-Related Lymphoproliferations: What’s New?
1 finding
Classification of B-Cell Lymphomas and Immunodeficiency-Related Lymphoproliferations: What’s New?
"New insights from genomic studies have had an impact on the definition and the diagnosis of several lymphoid tumors including follicular B-cell lymphomas, aggressive diffuse large B-cell lymphomas, and lymphoproliferations associated with acquired and posttransplant immunodeficiencies."
Show evidence (1 reference)
"New insights from genomic studies have had an impact on the definition and the diagnosis of several lymphoid tumors including follicular B-cell lymphomas, aggressive diffuse large B-cell lymphomas, and lymphoproliferations associated with acquired and posttransplant immunodeficiencies."
Deep research cited this publication as relevant literature for Follicular Lymphoma.
Advances in the multi-omics landscape of follicular lymphoma
1 finding
Advances in the multi-omics landscape of follicular lymphoma
"Advances in the multi-omics landscape of follicular lymphoma"

Deep Research

1
Falcon
WHO‑HAEM5 Subtype Schema (visual evidence)
Edison Scientific Literature 28 citations 2026-05-08T16:36:36.871015

1. Disease Information

Concise overview (current understanding)

Follicular lymphoma is an indolent, GC B‑cell–derived lymphoid neoplasm characterized by a follicular (nodular) architecture composed of centrocytes and centroblasts, typically presenting with diffuse lymphadenopathy and frequent bone marrow involvement, with a long relapsing–remitting course (jacobsen2022follicularlymphoma2023 pages 1-2, xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 2-4).

Abstract quote (definition/heterogeneity): “Follicular lymphoma (FL) is the most common indolent lymphoma originating from germinal center B cells. FL represents a clinically and biologically heterogeneous disease.” (Xu et al., published 2023‑03‑27; doi:10.7150/ijbs.80401) (xu2023advancesinthe pages 1-2).

Key identifiers (available in retrieved sources)

  • ICD‑9‑CM: 202.0 (used to identify FL in an Italian administrative-database real‑world evidence study) (Ferreri et al., published 2023‑09‑02; doi:10.3390/cancers15174403) (ferreri2023burdenofillness pages 2-3).
  • ICD‑10/ICD‑11, MeSH, Orphanet, MONDO: Not explicitly provided in the retrieved texts; these require direct database queries (OMIM/Orphanet/MeSH/MONDO/ICD) outside the accessed excerpts.

Synonyms / alternative names (from classification context)

  • Classic follicular lymphoma (cFL) (WHO‑HAEM5 term encompassing prior FL grades 1–2–3A) (Kurz et al., 2023‑01; doi:10.3390/cancers15030785) (kurz2023follicularlymphomain pages 2-4, kurz2023follicularlymphomain pages 4-6).
  • Follicular large B‑cell lymphoma (FLBCL) (WHO‑HAEM5 term replacing former grade 3B FL) (kurz2023follicularlymphomain pages 1-2, laurent2023follicularlymphomaand pages 1-2).
  • BCL2‑rearrangement–negative, CD23‑positive follicle center lymphoma (ICC provisional entity; overlaps with predominantly diffuse FL) (kurz2023follicularlymphomain pages 8-10, chadburn2023classificationofbcell pages 2-5, laurent2023follicularlymphomaand pages 1-2).

Evidence sources (patient-level vs aggregated)

The report draws from both aggregated disease-level resources (WHO‑HAEM5/ICC classification reviews; multi‑omics review; therapy landscape reviews) and patient-level cohorts/registries including SEER registry analyses and administrative real‑world evidence (RWE) datasets (kurz2023follicularlymphomain pages 1-2, xu2023advancesinthe pages 1-2, vaughn2023survivalofpatients pages 1-2, ferreri2023burdenofillness pages 1-2).


2. Etiology

Disease causal factors (mechanistic)

FL pathogenesis is widely described as a multistep process where an initiating lesion (often IGH::BCL2 t(14;18)) provides a survival advantage, followed by accumulation of additional genetic/epigenetic and microenvironment‑mediated events (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 4-6).

Genetic risk factors (susceptibility)

The retrieved sources note familial aggregation: “The incidence is slightly increased among relatives of persons with FL.” (Jacobsen, 2022‑10; doi:10.1002/ajh.26737) (jacobsen2022follicularlymphoma2023 pages 1-2).

No GWAS loci or germline pathogenic variants were captured in the retrieved excerpts; these would require dedicated searches (GWAS Catalog/ClinVar/ClinGen).

Environmental / infectious risk factors

No validated environmental or infectious causal factors for FL were provided in the retrieved excerpts. (Some literature exists for pesticides/immune dysregulation, but it was not accessible in the current evidence set.)

Protective factors / gene–environment interactions

Not identified in the retrieved evidence.


3. Phenotypes (clinical features)

Common presentation and manifestations

  • Lymphadenopathy and often bone marrow involvement; splenomegaly is also common (jacobsen2022follicularlymphoma2023 pages 1-2, kurz2023follicularlymphomain pages 2-4).
  • Cytopenias can occur, while classic “B symptoms” are less common unless transformation occurs (as summarized in Jacobsen’s clinical overview) (jacobsen2022follicularlymphoma2023 pages 1-2).
  • Transformation to DLBCL can present with rapid progression of lymphadenopathy, extranodal disease, B symptoms, hypercalcemia, and elevated LDH (jacobsen2022follicularlymphoma2023 pages 1-2).

Temporal features

  • Typical diagnosis in older adults: median age ~65 years (jacobsen2022follicularlymphoma2023 pages 1-2).
  • Transformation risk reported as about 2% per year in classification and clinical updates (jacobsen2022follicularlymphoma2023 pages 1-2, kurz2023follicularlymphomain pages 2-4).

Quality of life (QoL) impact (real-world data)

In an international RWE survey using EORTC QLQ‑C30, QoL worsened with later lines of therapy (LOT): mean global health status/QoL declined from 56.5 in 1L to 50.4 in 3L+ (Johnson et al., received 2024‑02‑23; published online 2024‑07‑08; doi:10.1007/s12325-024-02882-1) (johnson2024qualityoflife pages 1-2).

Suggested HPO terms (non-exhaustive; mapped to evidence above)

  • Lymphadenopathy (HP:0002716)
  • Splenomegaly (HP:0001744)
  • Bone marrow infiltration (HP:0005528) / Abnormal bone marrow morphology (HP:0005560)
  • Cytopenia (HP:0001875) (use specific: anemia HP:0001903, thrombocytopenia HP:0001873, neutropenia HP:0001875)
  • Fever (HP:0001945), Night sweats (HP:0030156), Weight loss (HP:0001824) — especially in transformation context
  • Elevated LDH (HP:0003077)

(Frequency estimates for specific phenotypes were not captured in the retrieved excerpts.)


4. Genetic / Molecular Information

Core disease-defining lesion

IGH::BCL2 t(14;18)(q32;q21) is the molecular hallmark of classic FL and is described as the initiating event in most cases (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 4-6, zinzani2024currentandfuture pages 1-2).

Recurrent somatic alterations (multi-omics/genomics)

High-frequency events involve epigenetic modifiers and immune–microenvironment modulators; examples include KMT2D, CREBBP, EZH2, EP300, and TNFRSF14 (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 4-6).

Variant biology (t(14;18)-negative, CD23+/STAT6)

WHO‑HAEM5 and ICC recognize variant subtypes, including a predominantly diffuse pattern often t(14;18)-negative, frequently CD23‑associated, and enriched for STAT6 mutations and 1p36 alterations involving TNFRSF14 (kurz2023follicularlymphomain pages 1-2, kurz2023follicularlymphomain pages 8-10, chadburn2023classificationofbcell pages 2-5).

Somatic vs germline

The alterations summarized here are described in the literature as somatic tumor events; germline predisposition variants were not provided in the retrieved excerpts.

Artifact: consolidated alteration table

The following table is structured for direct knowledge-base ingestion.

Alteration/Gene (HGNC symbol) Type (translocation/mutation/CNA/epigenetic) Approx frequency (with range) Functional role/pathway Clinical relevance (diagnostic/prognostic/therapeutic) Notes on subtype specificity (classic FL vs predominantly diffuse/CD23+ STAT6-mut, transformation) Key supporting sources (include DOI and year)
IGH::BCL2 / t(14;18)(q32;q21) Translocation ~65–90%; ~85% in classic FL Deregulated BCL2 anti-apoptotic signaling; founding lesion in germinal-center B cells Hallmark diagnostic lesion for classic FL; helps distinguish classic FL from several t(14;18)-negative variants; biologic rationale for FL pathogenesis Enriched in classic FL; usually absent in predominantly diffuse/CD23+ FL and other t(14;18)-negative variants; present in ISFN; pre-existing BCL2 translocation constrains some transformed aggressive lymphomas Xu 2023 doi:10.7150/ijbs.80401; Kurz 2023 doi:10.3390/cancers15030785; Jacobsen 2022 doi:10.1002/ajh.26737 (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 2-4, kurz2023follicularlymphomain pages 4-6, jacobsen2022follicularlymphoma2023 pages 1-2)
BCL2 Mutation / overexpression Mutated ~40% in one genomic cohort; protein expression ~80–90% in classic FL Anti-apoptotic mitochondrial pathway Diagnostic support via BCL2 protein expression in classic FL; central pathogenic driver; potential relevance to therapy resistance biology Strong expression typical of classic FL; lower expression in FLBCL/former grade 3B; predominantly diffuse FL can be BCL2+ despite lacking IGH::BCL2 Mozas 2023 doi:10.1002/hon.3132; Kurz 2023 doi:10.3390/cancers15030785 (kurz2023follicularlymphomain pages 4-6, kurz2023follicularlymphomain pages 8-10)
KMT2D Mutation (epigenetic modifier) ~70–90%; 79% in one cohort Histone methylation/chromatin regulation in GC B cells Very early driver lesion; part of core FL genomic profile; potential biomarker for clonal origin and early detection/monitoring Common in classic FL and early precursor cells; identified as an early stable event longitudinally; also implicated in transformed/aggressive evolution broadly but not specific for diffuse/CD23+ subtype Xu 2023 doi:10.7150/ijbs.80401; Mozas 2023 doi:10.1002/hon.3132; Bai 2024 doi:10.1038/s41408-024-01124-5; Friedberg 2023 doi:10.1002/hon.3138 (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 4-6, friedberg2023updateonfollicular pages 1-3)
CREBBP Mutation / loss-of-function (epigenetic modifier) ~50–70%; 67% in one cohort Histone acetylation/transcriptional control; epigenetic regulation and immune interaction Very early driver lesion; part of core FL genomic profile; mechanistically linked to pathogenesis and risk stratification research Common in classic FL and precursor cells; also frequent in t(14;18)-negative predominantly diffuse/CD23+ FL; longitudinal data identify it as an early event, with KAT-domain-mutant cases showing lower transformation risk in one 2024 study Xu 2023 doi:10.7150/ijbs.80401; Mozas 2023 doi:10.1002/hon.3132; Bai 2024 doi:10.1038/s41408-024-01124-5; Kurz 2023 doi:10.3390/cancers15030785 (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 4-6, kurz2023follicularlymphomain pages 8-10)
EZH2 Gain-of-function mutation (epigenetic modifier) ~20–30%; ~25% often cited PRC2/H3K27 methylation; germinal-center epigenetic program Therapeutically actionable: basis for tazemetostat use; core FL biology and biomarker testing in relapsed disease Seen in classic FL; EZH2-mutant tumors show altered histone gene expression/H3K27me3 patterns; not a defining lesion of diffuse/CD23+ STAT6-mut subtype Xu 2023 doi:10.7150/ijbs.80401; Romero 2024 doi:10.1038/s41467-024-47701-x; Friedberg 2023 doi:10.1002/hon.3138; Zinzani 2024 doi:10.1186/s40164-024-00551-1 (xu2023advancesinthe pages 1-2, friedberg2023updateonfollicular pages 1-3, zinzani2024currentandfuture pages 1-2)
EP300 Mutation (epigenetic modifier) ~20–30% Histone acetylation/co-activator function Recurrent lesion supporting epigenetic dysregulation model; potential future therapeutic relevance Present across classic FL; may occur in common precursor cells; not highlighted as subtype-defining for diffuse/CD23+ disease in gathered evidence Xu 2023 doi:10.7150/ijbs.80401; Bai 2024 doi:10.1038/s41408-024-01124-5 (xu2023advancesinthe pages 1-2)
TNFRSF14 Mutation / 1p36 loss-associated lesion ~46% altered in one cohort Microenvironment modulation / immune-stromal interaction Recurrent early lesion; may inform biology of FL–microenvironment crosstalk and subtype assignment Common in classic FL precursor cells; particularly relevant in t(14;18)-negative predominantly diffuse/CD23+ FL where 1p36 deletion/TNFRSF14 alteration is characteristic Mozas 2023 doi:10.1002/hon.3132; Kurz 2023 doi:10.3390/cancers15030785 (kurz2023follicularlymphomain pages 4-6, kurz2023follicularlymphomain pages 8-10, kurz2023follicularlymphomain pages 1-2)
STAT6 Mutation Frequent in t(14;18)-negative CD23+ diffuse FL; exact range not uniformly given in gathered evidence JAK/STAT signaling; linked to nuclear phospho-STAT6 and anti-apoptotic targets such as BCL2L1/BCL-xL Useful subtype-defining biomarker for BCL2-rearrangement-negative, CD23-positive follicle center lymphoma / predominantly diffuse FL Characteristic of predominantly diffuse inguinal-region, CD23+ FL; more frequent in BCL2-negative / t(14;18)-negative variants than classic FL Kurz 2023 doi:10.3390/cancers15030785; Chadburn 2023 doi:10.3390/hemato4010003; Laurent 2023 doi:10.1007/s00428-022-03432-2 (kurz2023follicularlymphomain pages 8-10, kurz2023follicularlymphomain pages 1-2, chadburn2023classificationofbcell pages 2-5, laurent2023follicularlymphomaand pages 1-2)
1p36 deletion / CN-LOH Copy-number alteration Recurrent; exact overall frequency not uniform, but highlighted as characteristic in diffuse FL Often spans TNFRSF14; contributes to immune/microenvironment dysregulation Helpful in subtype recognition for predominantly diffuse/t(14;18)-negative FL; may have prognostic implications in variant disease Characteristic of predominantly diffuse/CD23+ STAT6-mut FL; less emphasized as defining lesion in classic FL Kurz 2023 doi:10.3390/cancers15030785 (kurz2023follicularlymphomain pages 8-10, kurz2023follicularlymphomain pages 1-2, kurz2023follicularlymphomain pages 4-6)
16p13 loss / CN-LOH Copy-number alteration Recurrent in diffuse FL; no uniform cohort-wide percentage in gathered evidence Region includes CREBBP, CIITA, SOCS1; epigenetic and immune-regulatory consequences Supports molecular classification of t(14;18)-negative predominantly diffuse FL Highlighted in predominantly diffuse/CD23+ FL rather than classic FL Kurz 2023 doi:10.3390/cancers15030785 (kurz2023follicularlymphomain pages 8-10)
Chromosome 18q21 gain / chromosome 18 gain Copy-number alteration 14% localized FL vs 36% systemic FL in one study; chromosome 18 gain identified as early stable event in longitudinal series May increase dosage of 18q genes including BCL2/TCF4-region–related drivers Potential prognostic marker; 18q21 gains associated with inferior PFS in localized FL More frequent in systemic than localized FL; early stable CNA in disease evolution Ott 2023 doi:10.21203/rs.3.rs-3073791/v1; Bai 2024 doi:10.1038/s41408-024-01124-5 (xu2023advancesinthe pages 1-2)
6q loss Copy-number alteration Recurrent early stable CNA; exact frequency not uniform in gathered evidence Tumor-suppressor region loss Candidate marker of early clonal architecture and disease monitoring Early and stable across longitudinal FL evolution; not specifically subtype-restricted in gathered evidence Bai 2024 doi:10.1038/s41408-024-01124-5 (xu2023advancesinthe pages 1-2)
BCL6 rearrangement Translocation ~10–20% in classic FL; ~20% MYC translocations noted in FLBCL, not BCL6 Germinal-center transcriptional control Supports molecular heterogeneity; may complicate differential diagnosis with aggressive B-cell lymphomas Present in subset of classic FL; variant/transformed cases may carry additional aggressive-genotype features Kurz 2023 doi:10.3390/cancers15030785 (kurz2023follicularlymphomain pages 4-6)
ARID1A Mutation 29% systemic FL vs 6% localized FL in one 2023 study Chromatin remodeling (SWI/SNF) Candidate marker of systemic disease biology; may relate to more advanced disease state Enriched in systemic vs localized FL; not a classic hallmark but useful for biologic stratification Ott 2023 doi:10.21203/rs.3.rs-3073791/v1 (xu2023advancesinthe pages 1-2)
FOXO1 / PIM1 / TMEM30A Mutations Adverse-behavior associations reported in small cohort; no stable population frequency established Signaling, proliferation, membrane biology Possible adverse prognostic markers, but evidence remains preliminary Associated with early relapse/POD24 or refractory behavior in a small genomic study; not yet established as subtype-defining Mozas 2023 doi:10.1002/hon.3132 (xu2023advancesinthe pages 1-2)
CARD11 / CD79B Mutations Recurrent but lower-frequency; exact range not uniform in gathered evidence B-cell receptor/NF-κB signaling Support activation of oncogenic signaling pathways; potential future targeted-therapy relevance Part of broader signaling lesions in FL; not emphasized as variant-specific in gathered evidence Xu 2023 doi:10.7150/ijbs.80401 (xu2023advancesinthe pages 1-2)
IGHV N-glycosylation motif acquisition Somatic hypermutation-related molecular feature ~80% Promotes microenvironmental interactions and B-cell receptor biology Pathobiologic hallmark rather than routine diagnostic biomarker; may help explain dependence on microenvironment Associated mainly with classic FL biology and germinal-center origin Xu 2023 doi:10.7150/ijbs.80401; Kurz 2023 doi:10.3390/cancers15030785 (xu2023advancesinthe pages 1-2, kurz2023follicularlymphomain pages 2-4)

Table: This table summarizes the principal genomic and molecular abnormalities reported for follicular lymphoma in the gathered evidence, including frequencies, pathways, clinical relevance, and subtype specificity. It is designed for direct use in a disease knowledge base and emphasizes distinctions between classic FL and t(14;18)-negative diffuse/CD23+/STAT6-mutant variants.


5. Environmental Information

No specific environmental/lifestyle/infectious contributors were established in the retrieved excerpts for FL onset or progression. The biology-focused reviews emphasize tumor microenvironment dependence rather than an infectious etiology (xu2023advancesinthe pages 1-2).


6. Mechanism / Pathophysiology

Multi-step lymphomagenesis and microenvironment

FL development is described as a multistep process in which BCL2-overexpressing precursor B cells accumulate additional lesions through repeated GC re‑entry; disease progression and immune escape are strongly shaped by tumor–microenvironment crosstalk (xu2023advancesinthe pages 1-2).

Abstract quote (microenvironment role): multi‑omics analyses provide “a comprehensive profile of microenvironmental components… unveiling the crosstalk between tumor and microenvironment that induce FL progression and facilitate immune escape.” (Xu et al., 2023‑03‑27; doi:10.7150/ijbs.80401) (xu2023advancesinthe pages 1-2).

Key molecular pathways highlighted

  • Apoptosis evasion via BCL2 overexpression after IGH::BCL2 translocation (xu2023advancesinthe pages 1-2, zinzani2024currentandfuture pages 1-2).
  • Epigenetic dysregulation via frequent mutations in histone modifiers/acetyltransferases (KMT2D, CREBBP, EZH2, EP300) (xu2023advancesinthe pages 1-2, zinzani2024currentandfuture pages 1-2).
  • B‑cell receptor signaling pathway activation (PI3K/BTK/SYK noted as frequently activated and therapeutic targets) (zinzani2024currentandfuture pages 1-2).
  • JAK/STAT signaling in STAT6‑mutant, CD23‑associated t(14;18)-negative diffuse FL variant (kurz2023follicularlymphomain pages 8-10).

Suggested GO biological process terms (examples)

  • Regulation of apoptotic process (GO:0042981)
  • Chromatin organization (GO:0006325)
  • Histone modification (GO:0016570)
  • B cell receptor signaling pathway (GO:0050853)
  • T cell activation (GO:0042110)
  • Antigen processing and presentation (GO:0019882) (relevant to immunotherapy biology)

Suggested Cell Ontology (CL) terms for key cell types in FL microenvironment

  • Germinal center B cell (CL:0002633)
  • T follicular helper cell (CL:0002038)
  • Regulatory T cell (CL:0000815)
  • CD8-positive, alpha-beta T cell (CL:0000625)
  • Macrophage (CL:0000235)
  • Follicular dendritic cell (CL:0000447)

7. Anatomical Structures Affected

Organ/tissue distribution

  • Lymph nodes (primary; often disseminated) (xu2023advancesinthe pages 1-2).
  • Bone marrow involvement commonly noted (xu2023advancesinthe pages 1-2, jacobsen2022follicularlymphoma2023 pages 1-2).
  • Spleen involvement occurs (kurz2023follicularlymphomain pages 2-4).

Suggested UBERON terms (examples)

  • Lymph node (UBERON:0000029)
  • Bone marrow (UBERON:0002371)
  • Spleen (UBERON:0002106)

8. Temporal Development

Onset

FL is typically adult/older adult onset, with a reported median diagnostic age of ~65 years (jacobsen2022follicularlymphoma2023 pages 1-2).

Progression patterns

  • Indolent course with repeated remissions/relapses (johnson2024qualityoflife pages 1-2).
  • POD24 (progression within 24 months of frontline therapy) is emphasized as a high-risk temporal pattern with markedly worse OS (Zinzani et al., 2024‑08; doi:10.1186/s40164-024-00551-1) (zinzani2024currentandfuture pages 1-2).

Staging/progression to transformation

Histologic transformation to DLBCL is a key adverse event; one registry study identified biopsy‑confirmed transformation in 4.7% of FL patients at median follow-up 6.3 years (SEER 2010–2018) (Vaughn & Epperla, 2023‑09; doi:10.1186/s40364-023-00525-1) (vaughn2023survivalofpatients pages 1-2).


9. Inheritance and Population

Epidemiology (recent reviews)

  • FL is described as the second most common NHL subtype in Western settings (zinzani2024currentandfuture pages 1-2, xu2023advancesinthe pages 1-2).
  • Incidence: 2–4 per 100,000/year in Western countries (Zinzani et al., 2024‑08; doi:10.1186/s40164-024-00551-1) (zinzani2024currentandfuture pages 1-2).
  • Incidence (alternate estimate): 3–5 per 100,000/year (Xu et al., 2023‑03‑27; doi:10.7150/ijbs.80401) (xu2023advancesinthe pages 1-2).

Survival statistics

  • In rituximab era cohorts: 10‑year OS in French and US cohorts reported as 79.8% and 76.6% (Zinzani et al., 2024‑08) (zinzani2024currentandfuture pages 1-2).
  • Swedish registry (2003–2010): 10‑year OS by age group 18–49: 92%, 50–59: 83%, 60–69: 78%, ≥70: 64% (Jacobsen, 2022‑10; doi:10.1002/ajh.26737) (jacobsen2022follicularlymphoma2023 pages 1-2).

POD24 as a high-risk subgroup

Patients with POD24 had 2-year/5-year OS 68%/50% vs 97%/90% without POD24 (zinzani2024currentandfuture pages 1-2).


10. Diagnostics

Diagnostic approach (core elements)

  • Tissue biopsy with morphology showing closely packed neoplastic follicles is central, with WHO‑HAEM5/ICC emphasizing integration of morphologic, immunophenotypic, and molecular features (kurz2023follicularlymphomain pages 4-6, laurent2023follicularlymphomaand pages 1-2).
  • Immunophenotype (classic FL): pan‑B markers (CD19, CD20, PAX5) and GC markers (CD10, BCL6, HGAL); BCL2 protein expression in ~80–90% (often due to t(14;18)) (Kurz et al., 2023‑01; doi:10.3390/cancers15030785) (kurz2023follicularlymphomain pages 4-6).
  • Variant diagnostic hints: t(14;18)-negative, CD23-associated/diffuse-pattern FL variant enriched for STAT6 mutations and 1p36 lesions (kurz2023follicularlymphomain pages 8-10, kurz2023follicularlymphomain pages 1-2).

Classification visual evidence (WHO‑HAEM5 subtype list)

Table showing the WHO‑HAEM5 subtype schema was retrieved from Kurz et al. (2023) (kurz2023follicularlymphomain media 04784bfd).

Suggested molecular tests (based on current practice implications in retrieved sources)

  • FISH/NGS for IGH::BCL2 (t(14;18)) and sequencing of key epigenetic modifiers when clinically relevant (diagnosis/subtyping and targeted therapy decisions) (kurz2023follicularlymphomain pages 4-6, izutsu2024tazemetostatforrelapsedrefractory pages 1-2).

(Detailed imaging criteria, ctDNA/liquid biopsy validation, and differential diagnosis algorithms were not captured in the accessible excerpts.)


11. Outcome / Prognosis

Transformation prognosis (population data)

In a SEER analysis (2010–2018), transformed FL (t‑FL) had inferior outcomes compared with de novo DLBCL: - 5‑year relative survival 54% (t‑FL) vs 67% (de novo DLBCL) - 5‑year OS 49% vs 57% - Median OS 4.6 years vs 8.8 years** (Vaughn & Epperla, 2023‑09; doi:10.1186/s40364-023-00525-1) (vaughn2023survivalofpatients pages 1-2).

R/R FL outcomes benchmark

For patients with R/R FL after ≥2 prior therapies, a later-line benchmark reported median PFS 17 months and 5‑year OS 75% (Zinzani et al., 2024‑08) (zinzani2024currentandfuture pages 1-2).

Real-world mortality and cost burden (late-line RWE)

In an Italian administrative dataset of patients reaching ≥3 lines of therapy, 34% died at median 3‑year follow‑up; mean annual costs increased from €14,508 pre-inclusion to €21,081 at 1‑year follow-up (Ferreri et al., 2023‑09‑02; doi:10.3390/cancers15174403) (ferreri2023burdenofillness pages 1-2).


12. Treatment

Standard paradigms (high-level)

  • Many patients can be observed initially; systemic therapy can be deferred until symptoms or high tumor burden. Common systemic options include anti‑CD20–based regimens (rituximab ± chemotherapy) and chemo‑free immunomodulatory combinations (lenalidomide‑rituximab) (jacobsen2022follicularlymphoma2023 pages 1-2, ferreri2023burdenofillness pages 1-2).

2023–2024 advances and implementations in R/R FL

Novel immunotherapies (CD20×CD3 bispecific antibodies, CAR‑T) and targeted epigenetic therapy (EZH2 inhibition) are emphasized as major developments, with high response rates in later-line settings and expanding real-world uptake considerations (friedberg2023updateonfollicular pages 4-6, maurer2023matchingadjustedindirectcomparison pages 1-2, izutsu2024tazemetostatforrelapsedrefractory pages 1-2).

Artifact: therapy landscape summary table (quantitative)

Therapy (generic; target/class) Regulatory status (FDA/EMA/Japan approval year as stated) Key supporting study and design (trial name/NCT; publication year) Population/line Key efficacy (ORR, CR, PFS) and follow-up Key safety signals Real-world/health economics notes if present
Mosunetuzumab (CD20×CD3 bispecific antibody) FDA and European approvals in 2022 for R/R FL after ≥2 prior lines; FDA approval noted in Dec 2022 (friedberg2023updateonfollicular pages 4-6, jacobsen2022follicularlymphoma2023 pages 9-9) GO29781, pivotal international phase 2; phase I/II program; NCT02500407; reported in 2023–2024 contextual sources (friedberg2023updateonfollicular pages 4-6, maurer2023matchingadjustedindirectcomparison pages 1-2) R/R FL after ≥2 prior systemic therapies including alkylator and anti-CD20 therapy; high-risk subgroups included double-refractory and POD24 patients (friedberg2023updateonfollicular pages 4-6, maurer2023matchingadjustedindirectcomparison pages 1-2) Phase 2: ORR 80%, CR 60%, median PFS 17.9 months in GO29781; Friedberg update cites median follow-up >18 months, median PFS 21 months, median DOR 22 months; phase I data showed CR rates >50% in double-refractory and POD24 patients (maurer2023matchingadjustedindirectcomparison pages 1-2, friedberg2023updateonfollicular pages 4-6) CRS 44% in phase 2, almost all low grade; only one grade 3 and one grade 4 CRS event reported; phase I included neutropenia and CRS with fever, 7% hospitalization, neurologic AEs uncommon (friedberg2023updateonfollicular pages 4-6, jacobsen2022follicularlymphoma2023 pages 9-9) MAIC versus LEO CReWE: weighted real-world cohort ORR 73%, CR 53%, 12-month PFS 60% vs mosunetuzumab trial 58%; Bayesian long-term modeling estimated median survival 11.6–17.0 years; US budget model found per-patient cumulative cost about $202,039 and small 3-year payer impact (maurer2023matchingadjustedindirectcomparison pages 1-2)
Epcoritamab (subcutaneous CD20×CD3 bispecific antibody) Not stated as approved in the specific evidence set used here; described as in development/clinical advance in Jacobsen 2022 snippet (jacobsen2022follicularlymphoma2023 pages 9-9) Phase I/II study; Jacobsen update cites early epcoritamab data (publication context 2022/2023) (jacobsen2022follicularlymphoma2023 pages 9-9) R/R FL, later-line setting (jacobsen2022follicularlymphoma2023 pages 9-9) ORR 90%, CR 50% in the cited phase I/II experience (jacobsen2022follicularlymphoma2023 pages 9-9) CRS 59%, all grade 1–2 in cited snippet (jacobsen2022follicularlymphoma2023 pages 9-9) No direct RWE or health-economic data in the provided evidence set (jacobsen2022follicularlymphoma2023 pages 9-9)
Glofitamab (CD20×CD3 bispecific antibody) No approval status stated in the selected evidence set (jacobsen2022follicularlymphoma2023 pages 9-9) Phase I/II study with obinutuzumab pre-treatment; cited in Jacobsen update (jacobsen2022follicularlymphoma2023 pages 9-9) FL in relapsed/refractory setting (jacobsen2022follicularlymphoma2023 pages 9-9) ORR 70.5%, CR 47.7% in FL (jacobsen2022follicularlymphoma2023 pages 9-9) Low incidence of grade 3 CRS/neurologic events (jacobsen2022follicularlymphoma2023 pages 9-9) No direct RWE or economic data in the provided evidence set (jacobsen2022follicularlymphoma2023 pages 9-9)
Axicabtagene ciloleucel / axi-cel (autologous anti-CD19 CAR-T) Approved in the United States for relapsed/refractory FL based on ZUMA-5; approval noted by 2023 update sources (friedberg2023updateonfollicular pages 4-6) ZUMA-5 phase 2; mature results summarized in 2023 review (friedberg2023updateonfollicular pages 4-6) R/R FL, generally after failure of monoclonal antibody and alkylator therapy; considered for high-risk situations including selected POD24 patients (friedberg2023updateonfollicular pages 4-6) In primary FL analysis: CR 79%; estimated 18-month PFS 66%; durable responses reported (friedberg2023updateonfollicular pages 4-6) AEs similar to prior CAR-T experience; some decrease in severity of CRS and neurologic toxicity versus aggressive lymphoma histologies (friedberg2023updateonfollicular pages 4-6) Propensity-matched comparison favored CAR-T over matched cohort, with median PFS not reached vs 12 months in comparator cohort; US cost-effectiveness model estimated +1.89 QALY vs mosunetuzumab and ICER $108,307/QALY (friedberg2023updateonfollicular pages 4-6)
Tisagenlecleucel / tisa-cel (autologous anti-CD19 CAR-T) Approved by the United States FDA for FL based on trial cited in 2023 review (friedberg2023updateonfollicular pages 4-6) ELARA-related experience summarized in 2023 review (friedberg2023updateonfollicular pages 4-6) FL patients who received CAR-T infusion in relapsed/refractory setting (friedberg2023updateonfollicular pages 4-6) CR 69%; 12-month PFS 67% (friedberg2023updateonfollicular pages 4-6) Safety profile consistent with prior experience; nearly 20% treated outpatient in the cited trial summary (friedberg2023updateonfollicular pages 4-6) No direct FL-specific cost data in the selected evidence set; sequencing relative to bispecific antibodies remains unresolved (friedberg2023updateonfollicular pages 4-6)
Tazemetostat (EZH2 inhibitor; oral epigenetic therapy) US accelerated approval in 2020 for EZH2-mutated R/R FL after ≥2 prior therapies, or EZH2 wild-type without satisfactory alternatives; Japan approval in 2021 for EZH2-mutated R/R FL (izutsu2024tazemetostatforrelapsedrefractory pages 1-2) Global phase II NCT01897571 and Japanese phase II NCT03456726; 3-year follow-up published 2024 (izutsu2024tazemetostatforrelapsedrefractory pages 1-2) EZH2-mutated R/R FL, generally third line or later (izutsu2024tazemetostatforrelapsedrefractory pages 1-2) Global phase II: ORR 69% in EZH2-mut cohort and 35% in wild-type cohort; Japanese 3-year follow-up: FL cohort ORR 70.6%, median PFS not reached, 24-month PFS 72.1%, 36-month PFS 64.1%, median follow-up 35.0 months (izutsu2024tazemetostatforrelapsedrefractory pages 1-2) Long-term safety favorable; newly emerged grade 1–2 urinary tract infection, peripheral motor neuropathy, hypogammaglobulinemia; no unexpected grade ≥3 treatment-related AEs (izutsu2024tazemetostatforrelapsedrefractory pages 1-2) Oral, time-continuous targeted option; useful for biomarker-selected EZH2-mutated patients; no direct RWE cost data in selected evidence set (izutsu2024tazemetostatforrelapsedrefractory pages 1-2)
Later-line R/R FL prognosis benchmark (context for novel therapies, not a therapy) Not applicable (zinzani2024currentandfuture pages 1-2) LEO CReWE real-world benchmark cited in 2024 review (zinzani2024currentandfuture pages 1-2) Patients with R/R FL after ≥2 prior therapies (zinzani2024currentandfuture pages 1-2) Median PFS 17 months and 5-year OS 75% in R/R FL after ≥2 prior therapies; POD24 subgroup had 2-year/5-year OS 68%/50% vs 97%/90% without POD24 (zinzani2024currentandfuture pages 1-2) Not applicable Useful comparator showing unmet need that newer bispecifics, CAR-T, and EZH2-targeted therapy aim to address (zinzani2024currentandfuture pages 1-2)

Table: This table summarizes major 2022-2024 therapeutic advances for relapsed/refractory follicular lymphoma using only the cited context, with trial-level efficacy, safety, and selected real-world or economic context. It is useful for comparing bispecific antibodies, CAR-T products, and EZH2-targeted therapy against the poor-outcome benchmark of later-line FL.

Example MAXO terms (suggestions)

  • Anti-CD20 monoclonal antibody therapy (MAXO:0000013; general antibody therapy category—exact MAXO mapping may need verification)
  • Chimeric antigen receptor T-cell therapy (CAR‑T; MAXO term should be selected from MAXO hierarchy)
  • Bispecific antibody therapy (MAXO mapping required)
  • Radiotherapy (MAXO mapping required)

(MAXO IDs require ontology lookup; not explicitly present in retrieved evidence.)


13. Prevention

Current state

No established primary prevention strategy was identified in the retrieved literature excerpts. Current “prevention” is largely tertiary (preventing relapse/complications via optimized therapy sequencing and supportive care) and secondary (monitoring for progression/transformation), but specific evidence-based screening programs for asymptomatic individuals were not captured in the retrieved excerpts.


14. Other Species / Natural Disease

Not addressed in the retrieved excerpts; would require targeted searches in OMIA/veterinary oncology and comparative pathology resources.


15. Model Organisms

No specific FL model organism systems were extracted from the retrieved excerpts (despite the general recognition that microenvironment modeling is a research focus). Dedicated searches for genetically engineered mouse models (e.g., BCL2 transgenic GC models), xenografts, and 3D co-culture systems are needed.


WHO‑HAEM5 Subtype Schema (visual evidence)

The WHO‑HAEM5 subtype list is shown in Kurz et al. Table 1 (kurz2023follicularlymphomain media 04784bfd).


Limitations of this report (evidence availability)

  1. Ontology identifiers (MONDO/MeSH/Orphanet/ICD‑10/ICD‑11) were not present in the accessed excerpts; only ICD‑9‑CM 202.0 was captured from an RWE study (ferreri2023burdenofillness pages 2-3).
  2. Etiology (environmental/infectious), protective factors, and gene–environment interactions were not covered by the accessible evidence set.
  3. Model organisms and non-human natural disease information was not retrieved.
  4. Some claims that typically require primary trial publications (e.g., full GO29781 paper) were supported here via accessible abstracts/reviews and an RWE comparator study; deeper extraction from full primary reports would strengthen a knowledge base entry (friedberg2023updateonfollicular pages 4-6, maurer2023matchingadjustedindirectcomparison pages 1-2).

References

  1. (xu2023advancesinthe pages 1-2): Tianyuan Xu, Zhong Zheng, and Weili Zhao. Advances in the multi-omics landscape of follicular lymphoma. International Journal of Biological Sciences, 19:1955-1967, Mar 2023. URL: https://doi.org/10.7150/ijbs.80401, doi:10.7150/ijbs.80401. This article has 10 citations and is from a peer-reviewed journal.

  2. (zinzani2024currentandfuture pages 1-2): Pier Luigi Zinzani, Javier Muñoz, and Judith Trotman. Current and future therapies for follicular lymphoma. Experimental Hematology & Oncology, Aug 2024. URL: https://doi.org/10.1186/s40164-024-00551-1, doi:10.1186/s40164-024-00551-1. This article has 28 citations and is from a peer-reviewed journal.

  3. (jacobsen2022follicularlymphoma2023 pages 1-2): Eric Jacobsen. Follicular lymphoma: 2023 update on diagnosis and management. American Journal of Hematology, 97:1638-1651, Oct 2022. URL: https://doi.org/10.1002/ajh.26737, doi:10.1002/ajh.26737. This article has 159 citations and is from a domain leading peer-reviewed journal.

  4. (kurz2023follicularlymphomain pages 2-4): Katrin S. Kurz, Sabrina Kalmbach, Michaela Ott, Annette M. Staiger, German Ott, and Heike Horn. Follicular lymphoma in the 5th edition of the who-classification of haematolymphoid neoplasms—updated classification and new biological data. Cancers, 15:785, Jan 2023. URL: https://doi.org/10.3390/cancers15030785, doi:10.3390/cancers15030785. This article has 53 citations.

  5. (ferreri2023burdenofillness pages 2-3): Andrés J. M. Ferreri, Pier Luigi Zinzani, Carlo Messina, Diletta Valsecchi, Maria Chiara Rendace, Eleonora Premoli, Elisa Giacomini, Chiara Veronesi, Luca Degli Esposti, and Paola Di Matteo. Burden of illness in follicular lymphoma with multiple lines of treatment, italian rwe analysis. Cancers, 15:4403, Sep 2023. URL: https://doi.org/10.3390/cancers15174403, doi:10.3390/cancers15174403. This article has 5 citations.

  6. (kurz2023follicularlymphomain pages 4-6): Katrin S. Kurz, Sabrina Kalmbach, Michaela Ott, Annette M. Staiger, German Ott, and Heike Horn. Follicular lymphoma in the 5th edition of the who-classification of haematolymphoid neoplasms—updated classification and new biological data. Cancers, 15:785, Jan 2023. URL: https://doi.org/10.3390/cancers15030785, doi:10.3390/cancers15030785. This article has 53 citations.

  7. (kurz2023follicularlymphomain pages 1-2): Katrin S. Kurz, Sabrina Kalmbach, Michaela Ott, Annette M. Staiger, German Ott, and Heike Horn. Follicular lymphoma in the 5th edition of the who-classification of haematolymphoid neoplasms—updated classification and new biological data. Cancers, 15:785, Jan 2023. URL: https://doi.org/10.3390/cancers15030785, doi:10.3390/cancers15030785. This article has 53 citations.

  8. (laurent2023follicularlymphomaand pages 1-2): Camille Laurent, James R. Cook, Tadashi Yoshino, Leticia Quintanilla-Martinez, and Elaine S. Jaffe. Follicular lymphoma and marginal zone lymphoma: how many diseases? Virchows Archiv, 482:149-162, Nov 2023. URL: https://doi.org/10.1007/s00428-022-03432-2, doi:10.1007/s00428-022-03432-2. This article has 70 citations and is from a peer-reviewed journal.

  9. (kurz2023follicularlymphomain pages 8-10): Katrin S. Kurz, Sabrina Kalmbach, Michaela Ott, Annette M. Staiger, German Ott, and Heike Horn. Follicular lymphoma in the 5th edition of the who-classification of haematolymphoid neoplasms—updated classification and new biological data. Cancers, 15:785, Jan 2023. URL: https://doi.org/10.3390/cancers15030785, doi:10.3390/cancers15030785. This article has 53 citations.

  10. (chadburn2023classificationofbcell pages 2-5): Amy Chadburn, Annunziata Gloghini, and Antonino Carbone. Classification of b-cell lymphomas and immunodeficiency-related lymphoproliferations: what’s new? Hemato, 4:26-41, Jan 2023. URL: https://doi.org/10.3390/hemato4010003, doi:10.3390/hemato4010003. This article has 6 citations.

  11. (vaughn2023survivalofpatients pages 1-2): John L. Vaughn and Narendranath Epperla. Survival of patients with transformed follicular lymphoma in the united states: a multiple cohort study. Biomarker Research, Sep 2023. URL: https://doi.org/10.1186/s40364-023-00525-1, doi:10.1186/s40364-023-00525-1. This article has 15 citations and is from a peer-reviewed journal.

  12. (ferreri2023burdenofillness pages 1-2): Andrés J. M. Ferreri, Pier Luigi Zinzani, Carlo Messina, Diletta Valsecchi, Maria Chiara Rendace, Eleonora Premoli, Elisa Giacomini, Chiara Veronesi, Luca Degli Esposti, and Paola Di Matteo. Burden of illness in follicular lymphoma with multiple lines of treatment, italian rwe analysis. Cancers, 15:4403, Sep 2023. URL: https://doi.org/10.3390/cancers15174403, doi:10.3390/cancers15174403. This article has 5 citations.

  13. (johnson2024qualityoflife pages 1-2): Patrick Connor Johnson, Abigail Bailey, Qiufei Ma, Neil Milloy, Emilia Biondi, Ruben G. W. Quek, Sarah Weatherby, and Sophie Barlow. Quality of life evaluation in patients with follicular cell lymphoma: a real-world study in europe and the united states. Advances in Therapy, 41:3342-3361, Jul 2024. URL: https://doi.org/10.1007/s12325-024-02882-1, doi:10.1007/s12325-024-02882-1. This article has 13 citations and is from a peer-reviewed journal.

  14. (friedberg2023updateonfollicular pages 1-3): Jonathan W. Friedberg. Update on follicular lymphoma. Hematological Oncology, 41:43-47, Jun 2023. URL: https://doi.org/10.1002/hon.3138, doi:10.1002/hon.3138. This article has 32 citations and is from a peer-reviewed journal.

  15. (kurz2023follicularlymphomain media 04784bfd): Katrin S. Kurz, Sabrina Kalmbach, Michaela Ott, Annette M. Staiger, German Ott, and Heike Horn. Follicular lymphoma in the 5th edition of the who-classification of haematolymphoid neoplasms—updated classification and new biological data. Cancers, 15:785, Jan 2023. URL: https://doi.org/10.3390/cancers15030785, doi:10.3390/cancers15030785. This article has 53 citations.

  16. (izutsu2024tazemetostatforrelapsedrefractory pages 1-2): Koji Izutsu, Kiyoshi Ando, Momoko Nishikori, Hirohiko Shibayama, Hideki Goto, Junya Kuroda, Koji Kato, Yoshitaka Imaizumi, Kisato Nosaka, Rika Sakai, Maho Abe, Seiichiro Hojo, Tadashi Nakanishi, and Shinya Rai. Tazemetostat for relapsed/refractory b-cell non-hodgkin lymphoma with ezh2 mutation in japan: 3-year follow-up for a phase ii study. International Journal of Hematology, 120:621-630, Aug 2024. URL: https://doi.org/10.1007/s12185-024-03834-9, doi:10.1007/s12185-024-03834-9. This article has 10 citations and is from a peer-reviewed journal.

  17. (friedberg2023updateonfollicular pages 4-6): Jonathan W. Friedberg. Update on follicular lymphoma. Hematological Oncology, 41:43-47, Jun 2023. URL: https://doi.org/10.1002/hon.3138, doi:10.1002/hon.3138. This article has 32 citations and is from a peer-reviewed journal.

  18. (maurer2023matchingadjustedindirectcomparison pages 1-2): Matthew J. Maurer, Carla Casulo, Melissa C. Larson, Thomas M. Habermann, Izidore S. Lossos, Yucai Wang, Loretta J. Nastoupil, Christopher Strouse, Dai Chihara, Peter Martin, Jonathon B. Cohen, Brad S. Kahl, W Richard Burack, Jean L. Koff, Yong Mun, Anthony Masaquel, Mei Wu, Michael C. Wei, Ashwini Shewade, Jia Li, James R. Cerhan, Brian K. Link, and Christopher R. Flowers. Matching-adjusted indirect comparison from the lymphoma epidemiology of outcomes consortium for real world evidence (leo crewe) study to a clinical trial of mosunetuzumab in relapsed or refractory follicular lymphoma. Haematologica, 109:2177-2185, Nov 2023. URL: https://doi.org/10.3324/haematol.2023.283737, doi:10.3324/haematol.2023.283737. This article has 9 citations.

  19. (jacobsen2022follicularlymphoma2023 pages 9-9): Eric Jacobsen. Follicular lymphoma: 2023 update on diagnosis and management. American Journal of Hematology, 97:1638-1651, Oct 2022. URL: https://doi.org/10.1002/ajh.26737, doi:10.1002/ajh.26737. This article has 159 citations and is from a domain leading peer-reviewed journal.