불응성 용혈성 빈혈을 지닌 소아 전신홍반루푸스에서의 Rituximab 치료 1례

저자들은 재발성, 난치성 용혈성 빈혈과 신세뇨관산증을 동반한 소아 전신홍반루프스 환아에 있어 rituximab 사용후 장기간의 완전 관해를 보인 증례를 경험하였고, 현재까지 국내에 보고된 바가 없기에 문헌고찰과 함께 보고하는 바이다. 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.