Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features

Fifty-two cases of autoimmune hemolytic anemia (AHA) were observed within a series of 1203 patients (4.3%) with chronic lymphocytic leukemia (CLL) fol lowed at a single institution. Nineteen were observed at the time of CLL di agnosis and 33 during the clinical follow-up, Ninety percent of the patient s with CLL/AHA showed active CLL and 25% had been treated previously, The a ntierythrocyte autoantibody (AeAb) was an IgG in 87% of cases and an IgM in 13%. A lymphocyte count more than 60 x 10(9)/L(P <.00001), age above 65 ye ars (P <.01), and male gender (P <.01) emerged as independent parameters th at correlated significantly with an increased rate of AHA at CLL diagnosis, Patients previously treated with chlorambucil (CB) plus prednisone (PDN) a nd with fludarabine plus PDN showed a similar rate of AHA (1.8% and 2.5%, r espectively). After steroid therapy associated with CB in case of active CL L, 70% of patients achieved the complete disappearance of the AeAb, The act uarial AHA relapse-free survival probability was 54% at 5 years and the med ian survival probability after AHA was 41 months, Infections represented th e main cause of morbidity and mortality. IgG AHA and the occurrence of AHA at the same time of CLL diagnosis emerged as independent factors significan tly correlated with a better survival probability of AHA/CLL patients, Take n together, this study indicates that in CLL, AHA is a rare event with no i ndependent effect on survival for which steroids, associated with CB if req uired, and a careful management of infections may successfully control the 2 conditions. Cooperative studies are needed to better define the optimal s teroid schedule and the therapeutic role of other immunosuppressive agents and splenectomy, (Blood, 2000;95:2786-2792) (C) 2000 by The American Societ y of Hematology.