Autoimmune hemolytic anemia (AIHA) is a relatively com-mon cause of anemia in children and adults with systemic lupus erythematosus (SLE). Although AIHA responds to steroids, in case of refractory or steroid-dependent AIHA, immunosuppressants and intravenous immunoglobulin have been used as second line agents. Rituximab, an anti-CD20 monoclonal antibody, is emerging in the treatment of SLE refractory to conventional therapy. Herein, we report a case of delayed and sustained remission of refractory hemolytic anemia in a child with SLE, post rituximab treatment. A 12-year-old female child with dizziness was referred to our department and was diagnosed with SLE combined with he-molytic anemia and renal tubular acidosis. Since frequent relapse of hemolytic anemia had occurred during the ste-roid tapering course, even though she had been treated with additional immunosuppressants (azathioprine, mycopheno-late mofetil), the patient received 2 doses of rituximab 500 mg at 2 weeks interval at 18 months post diagnosis. After 15 months of rituximab administration, her anemia and re-nal tubular acidosis were fully recovered, enough to stop all medications. She remained well without recurrence for up to 3 years and 4 months after rituximab treatment.

Keywords: Systemic lupus erythematosus, Hemolytic ane-mia, Rituximab, Steroid