Richter’s Syndrome: A Terminal Complication of Chronic Lymphocytic Leukemia with Distinct Clinicopathologic Features

Abstract
Long, John C., and Aisenberg, Alan C.: Richter’s syndrome. A terminal complication of chronic lymphocytic leukemia with distinct clinicopathologic features. Am J Clin Pathol 63: 786–795, 1975. Two patients with chronic lymphocytic leukemia of 8 and 9 years’ duration had a terminal illness characterized by weight loss, persistent fever, lymphadenopathy, and dysglobulinemia. In both cases autopsy revealed a pleomorphic histiocytic lymphoma which contained multinucleate tumor cells and was associated with persistent chronic lymphocytic leukemia. These two cases are examples of Richter’s syndrome, a clinically distinct complication of chronic lymphocytic leukemia that may be confused pathologically with Hodgkin’s disease. Review of the medical literature suggests that this syndrome has frequently gone unrecognized.

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