MYC/BCL2 double-hit high-grade B-cell lymphoma.

Journal Article (Journal Article;Review)

Double-hit lymphoma (DHL) has been defined by others as a B-cell lymphoma with MYC/8q24 rearrangement in combination with a translocation involving another gene, such as BCL2, BCL3, or BCL6. The most common form of DHL has translocations involving MYC and BCL2, also known as MYC/BCL2 DHL. In recent years, a number of case series of MYC/BCL2 DHL have been published. Most cases of MYC/BCL2 DHL morphologically resemble diffuse large B-cell lymphoma (DLBCL) or B-cell lymphoma, unclassifiable, with features intermediate between DLBCL and Burkitt lymphoma. These tumors are of B-cell lineage, have a germinal center B-cell immunophenotype with a high proliferation rate, and a complex karyotype. Patients with these tumors have an aggressive clinical course and poor prognosis despite high-intensity chemotherapy. More recently, studies have suggested expanding the spectrum of MYC/BCL2 DHL to include cases that have concurrent MYC and BCL2 cytogenetic abnormalities, but not necessarily translocations. In addition, overexpression of MYC and BCL2 has been shown in an appreciable subset of DLBCL tumors. These tumors show overlap with MYC/BCL2 DHL, but are not equivalent. In this review, we discuss the clinicopathologic, immunophenotypic, cytogenetic, and prognostic features of MYC/BCL2 DHL.

Full Text

Duke Authors

Cited Authors

  • Li, S; Lin, P; Young, KH; Kanagal-Shamanna, R; Yin, CC; Medeiros, LJ

Published Date

  • September 2013

Published In

Volume / Issue

  • 20 / 5

Start / End Page

  • 315 - 326

PubMed ID

  • 23939148

Electronic International Standard Serial Number (EISSN)

  • 1533-4031

Digital Object Identifier (DOI)

  • 10.1097/PAP.0b013e3182a289f2

Language

  • eng

Conference Location

  • United States