HODGKINS-DISEASE VARIANT OF RICHTERS-SYNDROME - EXPERIENCE AT A SINGLE INSTITUTION

Patients developing Hodgkin's disease (HD) after a diagnosis of chroni c lymphocytic leukemia (CLL), are frequently included in a series of p atients with Richter's syndrome (RS). We sought to determine the natur al history of the association of CLL and HD. Over a 21 year period, 13 74 patients with CLL have been registered in our computer data base. S even cases of CLL and HD have been documented and confirmed. The media n age of these patients was 71 years (range 44-77) and clinical featur es included male gender (86%), B symptomatology (86%), rapidly progres sive lymphadenopathy (71%), prior CLL therapy (71%), advanced Ann Arbo r stage (86%), marrow involvement with HD (43%), and autoimmune hemoly tic anemia (29%). HD was documented by excisional lymph node biopsy in six cases and splenectomy in one. Mixed cellularity HD was shown in s ix and nodular sclerosis in one. Five of the biopsies revealed interve ning areas consistent with small lymphocytic lymphoma. The Sternberg-R eed (SR) cells were CD15+ in 6/7 cases, and Ki-1+ in the 6 patients te sted. CD45 and CD20 staining of the SR cells was nonreactive. The medi an time to development of HD was 45 months (range 0 to 96). The overal l responses to different chemotherapy regimens was approximately 25% w ith only one CR. Six patients have died at 3, 9, 10, 13, 15 and 36 mon ths and one patient is alive with progressive disease at 11 months. Ou r data suggests that CLL patients have a heightened risk for HD, featu res of advanced HD on presentation, and a poor response rate with shor t survival.