Primary Cutaneous Diffuse Large B-Cell Lymphoma With a MYC-IGH Rearrangement and Gain of BCL2: Expanding the Spectrum of MYC/BCL2 Double-Hit Lymphomas.

TitlePrimary Cutaneous Diffuse Large B-Cell Lymphoma With a MYC-IGH Rearrangement and Gain of BCL2: Expanding the Spectrum of MYC/BCL2 Double-Hit Lymphomas.
Publication TypeJournal Article
Year of Publication2016
AuthorsTesto N, Olson LC, Subramaniyam S, Hanson T, Magro CM
JournalAm J Dermatopathol
Volume38
Issue10
Pagination769-74
Date Published2016 Oct
ISSN1533-0311
KeywordsAntineoplastic Combined Chemotherapy Protocols, Biomarkers, Tumor, Biopsy, Disease Progression, Fatal Outcome, Gene Rearrangement, Genes, Immunoglobulin Heavy Chain, Humans, Immunohistochemistry, In Situ Hybridization, Fluorescence, Lymphoma, Large B-Cell, Diffuse, Male, Middle Aged, Proto-Oncogene Proteins c-bcl-2, Proto-Oncogene Proteins c-myc, Skin Neoplasms, Time Factors
Abstract

Aggressive extracutaneous B-cell lymphomas span the various stages of B-cell ontogeny and include B-cell lymphoblastic lymphoma, Burkitt lymphoma, mantle cell lymphoma, and diffuse large B-cell lymphoma. Diffuse large B-cell lymphomas represent the most common histologic subtype of non-Hodgkin lymphomas, comprising 30% of adult non-Hodgkin lymphomas in the United States. A distinctive form of diffuse large B-cell lymphoma is the double-hit lymphoma, with most cases exhibiting a combined MYC and BCL2 rearrangement, leading some hematopathologists to propose the term MYC/BCL2 lymphoma. More recently, MYC rearrangement with multiple copies/gain of BCL2 or multiple copies/gain of MYC with a BCL2 rearrangement have been described and exhibit a very similar clinical course to conventional double-hit lymphomas. We report the seventh case of diffuse large B-cell lymphoma exhibiting this distinct cytogenetic abnormality and the first reported case in the skin. The patient's clinical course was aggressive, succumbing to disease 18 months after his initial presentation.

DOI10.1097/DAD.0000000000000607
Alternate JournalAm J Dermatopathol
PubMed ID27391453
Related Faculty: 
Cynthia M. Magro, M.D.

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