Comprehensive management of refractory autoimmune hemolytic anemia in pediatric beta-thalassemia major patient: A case report.

introductionand importance: Autoimmune hemolytic anemia (AIHA) is characterized by the production of antibodies against the red blood cells (RBCs) leading to increased hemolysis. Incidence of AIHA in patients with beta-thalassemia major is 5 of 100 thalassemia patients. CASE PRESENTATION: A 15-year-old female patient with refractory AIHA and thalassemia was unresponsive to steroid therapy. In this report, she was treated with cyclosporine and prednisone in addition to leucodepleted packed red cell (PRC). She was successfully got remission during one-year monitoring. CLINICAL DISCUSSION: Cyclosporine is an immunosuppressive agent that interferes with T-cells activation by inhibiting transcription of cytokines, such as interleukin 2 and interferon-ɣ. Cyclosporine and steroid have synergic effects to prevent antibody production. Nephrotoxicity is one of the most concerning effect in cyclosporine usage, but it rarely develops using doses lower than 5 mg/kgBW/day. In limited resources blood bank with ABO and Rh crossmatch only, leucodepleted PRC transfusion could be an effective way to prevent antibody formation to minor blood group.

conclusionCyclosporine and steroid could be considered for management in refractory AIHA with thalassemia patients. Non-pharmacological therapy such as leucodepleted PRC transfusion and limited donor transfusion could be considered.