Abstract:
Objective To investigate clinical characteristics of and efficacy of treatments for chronic lymphocytic leukemia (CLL) complicated with autoimmune hemolytic anemia (AIHA).
Methods Clinical data of 14 patients with CLL complicated with AIHA were retrospectively analyzed in Institute of Hematology & Blood Diseases Hospital from January 1989 to December 2013.
Results The study included 14 patients comprising 11 males and 3 females, and the median age of the patients was 57.5 years (range 42–69 years). Before treatment, four patients (28.6%) had symptom B, 9 (64.3%) had anemia, 10 (71.4%) had high lactate dehydrogenase (LDH) levels. After treatment with AIHA, 9 of the 14 patients were evaluated, 9 (100%) had high β2-microglobulin levels, and 3 (37.5%) had unmutated immunoglobulin heavy chain variable region (IGHV). The overall response rate (ORR) was 100% (9/9), and 6 (66.7%) patients had complete response (CR) , 3 (33.3%) had partial response (PR) . After treatment for CLL, 11 of the 14 patients were evaluated. The ORR was 63.6% (7/11). Three patients had CR (27.3%), 4 had PR (36.4%), 3 had stable disease (SD) (27.3%), and 1 had progressive disease (PD) (9.1%). In 11 patients with long-term follow-up data, the median PFS was 69 (4–120) months, and the median OS was 75 (4-128) months. The 5- and 10-year PFS were (62.3±15.0)% and (18.7±15.5)%, respectively, and the 5- and 10-year OS were (80.0±12.6)% and (24.0±19.5)%, respectively.
Conclusions AIHA is a common complication of CLL and can occur at different stages of CLL. Treatment with corticosteroids and rituximab combined with chemotherapy had a therapeutic effect in these patients, and the new targeted drugs are effective for CLL complicated with AIHA.