Richter syndrome
The transformation of chronic lymphocytic leukemia into an aggressive large cell lymphoma.
Full Definition
Richter syndrome, also known as Richter transformation, represents the evolution of chronic lymphocytic leukemia into a more aggressive lymphoma, typically diffuse large B-cell lymphoma. This transformation occurs in approximately 5-10% of CLL patients and is associated with rapidly enlarging lymphadenopathy, B symptoms, and elevated LDH. The prognosis is generally poor, with median survival measured in months rather than years. Diagnosis requires tissue biopsy demonstrating large cell morphology, and treatment typically involves intensive lymphoma-directed chemotherapy.
Usage
Usage note: Distinguish from other CLL progressions; always confirm with tissue biopsy when suspected.
In Context
- "PET scan revealed new hypermetabolic lymphadenopathy concerning for possible Richter syndrome." — Imaging interpretation report
- "Lymph node biopsy confirmed Richter syndrome with diffuse large B-cell lymphoma morphology." — Pathology report