Successful treatment of steroid refractory severe autoimmune hemolytic anemia with

Rituximab

T. Schanz, M. Hänel Chemnitz

Background: Autoimmune hemolytic anemia (AIHA) is a well-known complication of non-

Hodgkin's lymphoma (NHL) with steroids being the treatment of choice. However, therapy options

are limited in steroid refractory AIHA. Rituximab, a monoclonal anti-CD20 antibody, has been

recently used for treatment of refractory antibody mediated autoimmune diseases such as AIHA. Case

report: We report a 48-year-old female patient with immunocytoma presenting a Coombs positive

severe autoimmune hemolytic anemia caused by warm autoantibodies against CD3. Previous

treatments with steroids, azathioprine and cyclosporine had failed. At first presentation there was a

half-life of erythroctes of 7.6 days, the hemoglobin level was 3.5 mmol/l and a 15-fold elevation of

unconju-gated bilirubin was presented. The patient had a splenomegaly but no peripheral or

abdominal lymphoma was detected. The patient received Rituximab in dose of 375 mg/m2 once a

week for 4 weeks. After the second dose a decline in hemolysis parameters was observed leading to a

sustained stabilization of the hemoglobin value with no need of further treatment. This was

accompanied by a disappearance of the autoantibodies against CD3. With a follow-up of 4 weeks the

patient is free of hemolysis and in good condition. Conclusion: Rituximab appears to be a safe and

feasible treatment option in patients with steroid refractory AIHA.