Refractory Autoimmune Hemolytic Anemia in a Child with Beta thalassemia Major

Abstract

Autoimmune hemolytic anemia (AIHA) in Thalassemia major is a challenging entity. This is a case of 1 year old Thalassemia major patient who developed AIHA after few transfusions and became refractory to all available medical modalities. Before transplant, patient did not respond to high dose steroid therapy, two cycles (5 days) of Fludarabine- dexamethasone, two doses of Rituximab and IV immunoglobulin. After 100% HLA matched bone marrow transplant, patient remained stable till day +10, but than his AIHA worsened along with development of severe veno-occlusive disease. In addition to Cyclosporine and methotrexate he received high dose of steroids, Rituximab and IVIG with no response. He rejected to 100% HLA matched bone marrow transplant with persistent refractory AIHA. For refractory AIHA, the Proteasome inhibitor Bortezomib was also given. This case reflects that refractory AIHA associated with Beta Thalassemia can be a challenging clinical condition requiring new or redefined treatment modalities and it can adversely affect transplant outcome even in young patients undergoing 100% HLA matched bone marrow transplant