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Double hit lymphoma: contemporary understanding and practices.

Eashwar Somasundaram1, Jeremy S Abramson2

  • 1Department of Medicine, Massachusetts General Hospital, Boston, MA, USA.

Leukemia & Lymphoma
|October 8, 2024
PubMed
Summary

Double-hit lymphoma (DHL), characterized by MYC and BCL2 gene rearrangements, is an aggressive cancer. Current treatments include intensive chemotherapy, with emerging therapies like CAR-T cells offering new hope for relapsed patients.

Keywords:
BCL2DLBCLHGBCLHigh-grade B-cell lymphomaMYCdiffuse large B-cell lymphoma

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Area of Science:

  • Hematology
  • Oncology
  • Molecular Pathology

Background:

  • Double-hit lymphoma (DHL) is an aggressive subtype of large B-cell lymphoma.
  • It is defined by concurrent genetic rearrangements of the MYC and BCL2 genes.
  • DHL presents a diagnostic and therapeutic challenge due to its aggressive nature and varied morphology.

Purpose of the Study:

  • To provide a comprehensive review of Double-hit lymphoma.
  • To analyze the molecular basis, diagnostic criteria, prognostic indicators, and treatment strategies for DHL.
  • To synthesize recent literature on DHL for a better understanding of the disease.

Main Methods:

  • Histologic and immunophenotypic examination for diagnosis.
  • Fluorescence in situ hybridization (FISH) to confirm MYC and BCL2 rearrangements.
  • Literature review of recent studies on DHL.

Main Results:

  • DHL morphology can mimic diffuse large B-cell lymphoma (DLBCL) or Burkitt lymphoma.
  • Prognostic factors include elevated lactate dehydrogenase (LDH), advanced stage, and extranodal involvement.
  • Intensive chemotherapy regimens like dose-adjusted EPOCH-R show promising outcomes.
  • CAR-T cells and bispecific antibodies are emerging treatments for relapsed/refractory DHL.

Conclusions:

  • Accurate diagnosis of DHL relies on integrated histologic, immunophenotypic, and genetic analyses.
  • Prognosis is multifactorial, necessitating personalized treatment approaches.
  • Advances in immunotherapy offer new avenues for managing relapsed or refractory DHL.