High-grade B-Cell lymphoma with MYC and BCL6 rearrangements associated with Richter transformation of chronic lymphocytic leukemia
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.
Copyright (c) 2019 Autopsy and Case Reports
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Copyright and publishing license
Authors retain copyright granting the journal right of first publication with the work simultaneously licensed under the Creative Commons Attribution Non-Commercial License.
Autopsy and Case Reports accepted articles are published under the Creative Commons Attribution Non-Commercial License. Under this License, the authors agree to have the CC-BY-NC license applied to their work, which retains the author's ownership of the copyright for their article and permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original authors and source are properly cited. This facilitates freedom in re-use and also ensures that the journal's content can be mined without barriers for the needs of research.