Histologic Transformation of Indolent Lymphomas to DLBCL
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Questions and Answers

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:

<p>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</p> Signup and view all the answers

Match the following rates with their corresponding events:

<p>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</p> Signup and view all the answers

Match the following treatment strategies with the corresponding outcome:

<p>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</p> Signup and view all the answers

Match the following lymphomas with their genotype characteristics:

<p>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</p> Signup and view all the answers

Match the following outcomes with their corresponding lymphomas:

<p>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</p> Signup and view all the answers

Match the following lymphoma subtypes with the corresponding incidence of histologic transformation:

<p>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</p> Signup and view all the answers

Match the following cohorts with their corresponding studies:

<p>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</p> Signup and view all the answers

Match the following characteristics with the corresponding risk factor for histologic transformation:

<p>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</p> Signup and view all the answers

Match the following treatment outcomes with the corresponding prognosis:

<p>Complete response (CR) = Better prognosis Inability to achieve CR = Poor prognosis No previous exposure to chemotherapy = Improved survival High FLIPI scores = Poor prognosis</p> Signup and view all the answers

Match the following characteristics with the corresponding lymphoma subtype:

<p>Splenic MZL = Marginal zone lymphoma Nodal MZL = Marginal zone lymphoma Advanced stage FL = Follicular lymphoma TFL = Transformation follicular lymphoma</p> Signup and view all the answers

Match the following characteristics with the corresponding clinical outcome:

<p>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</p> Signup and view all the answers

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.

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Description

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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