Prolonged survival of a patient with Richter’s syndrome

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The development of a diffuse histiocytic (large cell) lymphoma as a “second” neoplasm complicates the course of chronic lymphocytic leukemia in less than 5% of cases.1–6 This condition has been termed Richter’s syndrome.7 Most patients die in a short time without achieving remissions.1, 2, 6 With improvement in chemotherapeutic regimens, an increasing number of patients with diffuse histiocytic lymphoma have achieved long-term, disease-free survivals.8, 9 We describe a patient who survived 10 years without evidence of disease following the treatment of Richter’s syndrome with chemotherapy and radiotherapy.

Case report

A 72-year-old white woman was found to have chronic lymphocytic leukemia at the age of 48. Laboratory studies at another hospital disclosed the following values: white blood count (WBC), 50 × 109/L; hemoglobin, 10 g/dl; and normal platelet count. Her initial treatment at the other institution included radiation therapy to sites of lymphadenopathy and to the spleen. She was first studied at the Cleveland Clinic when she was 50 years old. Bone marrow aspiration at that time showed an overall hyper-cellularity with 86% mature lymphocytes. No residual erythroid precursors were noted at that time. A cervical lymph node biopsy specimen was interpreted as lympho-sarcoma. She was again treated with splenic irradiation, intermittent infusions of normal plasma, and eventually with chlorambucil and prednisone, the latter being continued for approximately 4 years. During this time she was symptom free and her blood count gradually became normal; hemoglobin, 12 g/dl; WBC, 6.7 × 109/L with a normal differential and normal platelet count. She . . .



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