Autoimmune hemolytic anemia (AIHA) is a condition associated with an extensive differential diagnosis that includes lymphoid malignancies. Although AIHA occurs in about 10-25% of patients with chronic lymphocytic leukemia, it is also reported to occur in all of the other lymphoid subtypes. In this article, we report a case of recurrent AIHA in a 67-year-old woman with two acute episodes of hemolysis separated by 3 years of hematologic remission. Both episodes were severe enough to require blood transfusion, oral steroids, and rituximab. Bone marrow biopsy and immunophenotyping using flow cytometry done during both admissions confirmed the presence of splenic marginal zone lymphoma.
Keywords: Rituximab; autoimmune hemolytic anemia; prednisone; splenectomy; splenic marginal zone lymphoma.