Waldenstrom macroglobulinemia, which is characterized by elevation of serum monoclonal IgM paraprotein, has recently been responsible for the treatment of purine analogues. Fludarabine, one of purine analogues, has been associated with autoimmune hemolytic anemia in patient with chronic lymphocytic leukemia. However, autoimmune hemolytic anemia after fludarabine treatment for Waldenstrom macroglobulinemia has not been reported. We experienced a case of autoimmune hemolytic anemia after fludarabine treatment for Waldenstrom macroglobulinemia. In this case, hemolytic anemia with positive direct Coombs' test occurred at 20 months after the first administration of fludarabine, relapsed at 5 days after re-administration. This anemia responded to steroid therapy. Autoimmune hemolytic anemia associated with fludarabine therapy can be severe and fatal, especially if a patient is re-treated with this drug after a previous episode of hemolytic anemia.

Keywords: Autoimmune hemolytic anemia, Waldenstrom macroglobulinemia, Fludarabine