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.
Highlights
Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma
HGBL comprises a group of aggressive, mature B-cell lymphomas that can be further categorized as either “High-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements” or “High-grade B-cell lymphoma, NOS.”[1]. Most cases belong in the former category, and were previously referred to as
Prior to the 2016 revision to the WHO Classification of Hematopoietic and Lymphoid Tissues, these malignancies were classified as “B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma.”. These lymphomas typically occur in elderly patients and present at an advanced stage
Summary
Richter transformation (RT), or Richter syndrome, is defined as the transformation of chronic lymphocytic leukemia (CLL) to an aggressive B-cell lymphoma. Keywords Leukemia, Lymphocytic, Chronic, B-cell; Lymphoma, Non-Hodgkin; Lymphoma, Large B-cell, Diffuse; Cytogenetics
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