14 Questions
Match the following types of lymphomas with their characteristics:
FL = Indolent lymphoma DLBCL = Aggressive lymphoma HGBL = High-grade lymphoma with double-hit cytogenetics MZL = Marginal zone lymphoma
Match the following characteristics with the corresponding lymphoma subtype:
FL = Follicular lymphoma MZL = Marginal zone lymphoma DLBCL = Diffuse large B-cell lymphoma TFL = Transformation follicular lymphoma
Match the following entities with their classifications:
WHO-HAEM5 = Updated 2022 WHO classification of hematolymphoid tumors ICC = International Consensus Classification FL = Follicular lymphoma DLBCL = Diffuse large B-cell lymphoma
Match the following risk factors with the corresponding outcome:
Advanced stage FL = High risk of histologic transformation to DLBCL High FLIPI scores = Poor prognosis Elevated LDH = High risk of histologic transformation to DLBCL No previous exposure to chemotherapy = Improved survival
Match the following rates with their corresponding events:
2% to 3% per year = Histologic transformation of FL to DLBCL or HGBL 50% at 2 years = Survival rate of HGBL with double-hit cytogenetics 73% at 2 years = Survival rate of transformed FL with double-hit cytogenetics 77 of 140 deaths = Leading cause of death in patients with newly diagnosed FL
Match the following treatment strategies with the corresponding outcome:
Early initiation of treatment at diagnosis = Decreased risk of transformation Immediate treatment with rituximab = No difference in time to transformation Observation = No difference in incidence of histologic transformation Rituximab therapy = Improved survival
Match the following lymphomas with their genotype characteristics:
HGBL = Translocations of MYC with BCL2 or BCL6 rearrangement FL = No translocations of MYC DLBCL = Translocations of MYC with BCL6 rearrangement MZL = Translocations of MYC with BCL2 rearrangement
Match the following outcomes with their corresponding lymphomas:
Poor clinical outcome = Histologic transformation of FL to DLBCL or HGBL Inferior survival = HGBL with double-hit cytogenetics Leading cause of death = Histologically transformed FL Good clinical outcome = Indolent lymphoma
Match the following lymphoma subtypes with the corresponding incidence of histologic transformation:
FL = Higher incidence of histologic transformation to DLBCL MZL = Lower incidence of histologic transformation to DLBCL DLBCL = outcome of histologic transformation TFL = Transformation from FL to DLBCL
Match the following cohorts with their corresponding studies:
French and U.S. cohorts = Pooled analysis of patients with newly diagnosed FL European cohort = Study of patients with HGBL Asian cohort = Study of patients with MZL North American cohort = Study of patients with DLBCL
Match the following characteristics with the corresponding risk factor for histologic transformation:
Nodal MZL subtype = Higher incidence of histologic transformation to DLBCL High-risk IPI scores = Risk factor for histologic transformation to DLBCL Elevated LDH = Risk factor for histologic transformation to DLBCL Multiple mucosal site involvement = Risk factor for histologic transformation to DLBCL
Match the following treatment outcomes with the corresponding prognosis:
Complete response (CR) = Better prognosis Inability to achieve CR = Poor prognosis No previous exposure to chemotherapy = Improved survival High FLIPI scores = Poor prognosis
Match the following characteristics with the corresponding lymphoma subtype:
Splenic MZL = Marginal zone lymphoma Nodal MZL = Marginal zone lymphoma Advanced stage FL = Follicular lymphoma TFL = Transformation follicular lymphoma
Match the following characteristics with the corresponding clinical outcome:
Early-stage FL = Improved survival High-risk IPI scores = Poor prognosis B symptoms at diagnosis = Risk factor for histologic transformation to DLBCL Histologic transformation to DLBCL = Poor clinical outcome
Study Notes
Histologic Transformation of Indolent Lymphomas to DLBCL
- Histologic transformation of indolent lymphomas (follicular lymphoma [FL] or marginal zone lymphoma [MZL]) to diffuse large B-cell lymphoma (DLBCL) is recognized as an entity in the 2022 WHO classification of hematolymphoid tumors (WHO-HAEM5).
Risk Factors for Histologic Transformation of FL to DLBCL
- Advanced stage FL, high-risk FLIPI, and IPI scores at diagnosis increase the risk of histologic transformation.
- Elevated LDH and B symptoms at diagnosis are associated with a higher risk of transformation.
- Early initiation of treatment at diagnosis may decrease the risk of transformation.
Outcomes of Patients with Transformed FL (TFL)
- Histologic transformation of FL to DLBCL is associated with a poor clinical outcome.
- 2-year survival rates for transformed FL with double-hit cytogenetics are around 73%, compared to 50% for histologic transformation to high-grade B-cell lymphomas (HGBL) with double-hit cytogenetics.
- In the rituximab era, improved survival has been reported in a subset of patients, such as those with early-stage FL and no previous exposure to chemotherapy or rituximab prior to transformation.
Histologic Transformation of MZL to DLBCL
- The incidence of histologic transformation of MZL to DLBCL is lower than that of FL, but may occur across all subtypes.
- Nodal MZL subtype, high-risk IPI scores, elevated LDH, and B symptoms are associated with a higher risk of transformation.
- Inability to achieve complete response (CR) after initial treatment and disease involvement in multiple mucosal sites and >4 nodal sites at diagnosis are also risk factors for histologic transformation.
This quiz covers the histologic transformation of indolent lymphomas, such as follicular lymphoma or marginal zone lymphoma, to diffuse large B-cell lymphoma. It is a recognized entity in the updated 2022 WHO classification of hematolymphoid tumors.
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