Richter transformation
Pronunciation: RICK-ter
The transformation of chronic lymphocytic leukemia into a more aggressive lymphoma, typically diffuse large B-cell lymphoma.
Full Definition
Richter transformation, also known as Richter syndrome, refers to the evolution of chronic lymphocytic leukemia (CLL) into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL), occurring in approximately 5-10% of CLL patients. This transformation is characterized by rapid clinical deterioration with new or enlarging lymphadenopathy, systemic symptoms (fever, night sweats, weight loss), elevated lactate dehydrogenase, and hypercalcemia. The transformation may be clonally related to the original CLL clone or represent a de novo lymphoma. Prognosis is generally poor, with median survival measured in months, though some patients may benefit from intensive chemotherapy or clinical trial participation.
Usage
Usage note: Named after Maurice Richter. Should be distinguished from other forms of CLL progression such as prolymphocytic transformation.
In Context
- "PET scan findings were consistent with possible Richter transformation of the patient's chronic lymphocytic leukemia." — Imaging interpretation
- "Biopsy confirmed Richter transformation to diffuse large B-cell lymphoma." — Pathology report