Autopsy and Case Reports
https://www.autopsyandcasereports.org/article/doi/10.4322/acr.2019.090
Autopsy and Case Reports
Article / Clinical Case Report

High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia

Thomas S Rogers; Juli-Anne Gardner; Katherine A Devitt

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Abstract

Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma. The vast majority, up to 99%, transform into diffuse large B-cell lymphoma (DLBCL), with a small subset (<1%) becoming classical Hodgkin lymphoma. Approximately half of RT cases progress through a pathway involving dysregulation of C-MYC. High-grade B-cell lymphoma (HGBL) is a recent diagnostic category of aggressive B-cell lymphomas set forth in the updated 2017 WHO Classification of Hematopoietic and Lymphoid Tissues. HGBL with MYC and BCL2 and/or BCL6 rearrangements, formerly “double-hit” and “triple-hit” lymphomas, comprise the majority of HGBL cases. Patients with HGBL have a worse prognosis than those with diffuse large B-cell lymphoma. We present a case of RT with rearrangements of MYC and BCL6. To our knowledge, there are no reported cases of RT with a “double-hit” lymphoma genotype.

Keywords

Leukemia, Lymphocytic, Chronic, B-cell, Lymphoma, Non-Hodgkin, Lymphoma, Large B-cell, Diffuse, Cytogenetics

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Publication date:
07/19/2019

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