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How I treat double-hit lymphoma.
1James P. Wilmot Cancer Institute, University of Rochester, Rochester, NY.
Blood
|June 11, 2017
Summary
High-grade B-cell lymphomas with specific gene rearrangements, known as double-hit lymphomas, present a poor prognosis. Aggressive treatment regimens may improve outcomes for these aggressive lymphomas.
Area of Science:
- Hematology
- Oncology
- Genetics
Background:
- The 2016 WHO classification introduced a distinct category for high-grade B-cell lymphoma with specific gene rearrangements (MYC, BCL-2, BCL-6).
- These double-hit lymphomas (or triple-hit) are rare (<10% of diffuse large B-cell lymphoma) and distinct from double-expressor lymphomas identified by immunohistochemistry.
Purpose of the Study:
- To review the evaluation and treatment of double-hit lymphomas.
- To highlight the poor prognosis associated with standard chemoimmunotherapy and increased risk of CNS involvement.
Main Methods:
- Review of existing literature and retrospective studies on double-hit lymphomas.
- Discussion of diagnostic differentiation from double-expressor lymphomas using immunohistochemistry and genetic rearrangements.
Main Results:
- Double-hit lymphomas are associated with poor prognosis and increased risk of progression and CNS involvement.
- Retrospective data suggest aggressive induction regimens may lead to better outcomes.
Conclusions:
- Double-hit lymphomas require specific diagnostic criteria and treatment strategies.
- Future trials should explore aggressive regimens and targeted agents for improved patient outcomes.
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