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

• Published in: Autopsy & case reports Vol. 9; no. 3; p. e2019090
• Main Authors: Rogers, Thomas S, Gardner, Juli-Anne, Devitt, Katherine A
• Format: Journal Article
• Language: English
• Published: Brazil São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2019; University of São Paulo
• Subjects: Chronic, B-cell; Clinical Case Report; Large B-cell; Leukemia; Lymphocytic; Lymphoma; Non-Hodgkin; Lymphoma, Large B-cell, Diffuse; Cytogenetics; Lymphoma, Non-Hodgkin; Leukemia, Lymphocytic, Chronic, B-cell
• ISSN: 2236-1960; 2236-1960
• DOI: 10.4322/acr.2019.090

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
and
and/or
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
and
. To our knowledge, there are no reported cases of RT with a "double-hit" lymphoma genotype.