Delayed and Long-term Remission of Refractory Hemolytic Anemia in a Child with Systemic Lupus Erythematosus Treated with Rituximab

Autoimmune hemolytic anemia (AIHA) is a relatively common 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 hemolytic anemia and renal tubular acidosis. Since frequent relapse of hemolytic anemia had occurred during the steroid tapering course, even though she had been treated with additional immunosuppressants (azathioprine, mycophenolate 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 renal 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.