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Multiplexed Fluorescent Immunohistochemical Staining, Imaging, and Analysis in Histological Samples of Lymphoma
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MYC/BCL2 double-hit high-grade B-cell lymphoma.

Shaoying Li1, Pei Lin, Ken H Young

  • 1Department of Pathology, Microbiology, and Immunology, Division of Hematopathology, Vanderbilt University School of Medicine, Nashville, TN, USA.

Advances in Anatomic Pathology
|August 14, 2013
PubMed
Summary

Double-hit lymphoma (DHL) involves MYC gene rearrangements, often with BCL2. These aggressive B-cell lymphomas present challenges in diagnosis and treatment, despite intensive chemotherapy.

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Area of Science:

  • Hematology
  • Oncology
  • Genetics

Background:

  • Double-hit lymphoma (DHL) is characterized by MYC/8q24 rearrangement and a translocation involving another gene, commonly BCL2.
  • MYC/BCL2 DHL often presents with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma.
  • These B-cell neoplasms exhibit a germinal center B-cell immunophenotype, high proliferation rate, and complex karyotype.

Purpose of the Study:

  • To review the clinicopathologic, immunophenotypic, cytogenetic, and prognostic features of MYC/BCL2 DHL.
  • To discuss the expanding spectrum of MYC/BCL2 DHL, including cases with cytogenetic abnormalities beyond translocations.
  • To differentiate MYC/BCL2 DHL from DLBCL with MYC and BCL2 overexpression.

Main Methods:

  • Literature review of published case series and studies on MYC/BCL2 DHL.
  • Analysis of clinicopathologic, immunophenotypic, and cytogenetic data.
  • Comparison of features across different classifications of DHL and related B-cell lymphomas.

Main Results:

  • MYC/BCL2 DHL is an aggressive B-cell lymphoma with a poor prognosis despite intensive chemotherapy.
  • Morphological and immunophenotypic overlap exists between MYC/BCL2 DHL and DLBCL or B-cell lymphoma, unclassifiable.
  • Concurrent MYC and BCL2 cytogenetic abnormalities, including those without translocations, are increasingly recognized within the DHL spectrum.

Conclusions:

  • MYC/BCL2 DHL represents a distinct clinicopathologic entity with aggressive behavior.
  • Accurate diagnosis requires integration of morphological, immunophenotypic, and cytogenetic findings.
  • Further research is needed to optimize treatment strategies for patients with MYC/BCL2 DHL.