Chronic lymphocytic leukemia-associated autoimmune hemolytic anemia

The clinical course of patients with B-cell chronic lymphocytic leukemia (CLL) is often made complicated by autoimmune phenomena which mainly target the blood cells. Among them, the autoimmune hemolytic anemia ( AIHA) is the most common form. On the other hand, it is believed that CLL is the most common of the known causes of AIHA. The source of any putative autoantibody ( bystander nonmalignant cells or tumor cells) is not clear yet. Recently, it has been hypothesized that leukemic B-cells may also act as professional antigen presenting cells (APCs). With respect to the management of CLL-associated AIHA, steroids still represent the first-line treatment option. Intravenous immunoglobulin, immunosuppressive drugs, and splenectomy are also frequently used for steroid-refractory forms. Furthermore, although the case series is still too small, encouraging data is now supporting the use of monoclonal antibodies, in particular anti-CD20 rituximab, in managing this often life-threatening autoimmune complication of CLL.