Summary:Here, we report a case of paediatric -thalassaemia Patients with autoimmune haemolytic anaemia (AIHA) after allogeneic transplantation are usually treated with either steroids alone or multiple immunosuppressive therapies, including antithymocyte globulin, cyclophosphamide, immunoglobulin, splenectomy, vincristine and erythropoietin. The overall response to these therapies has been generally unsatisfactory.1 Quartier et al 2 described the treatment of childhood refractory AIHA with rituximab. Therefore, we would like to report our experience about a successful treatment of refractory AIHA in a post-unrelated allogeneic bone marrow transplant pediatric patient with rituximab.
Rituximab therapy for refractory AIHA post unrelated BMTA 37-month-old Thai boy who had -thalaessaemia major underwent unrelated T cell-nondepleted bone marrow transplantation. His course of transplant was uneventful with full donor engraftment. On day 180 post transplant, the child
Disarticulation of a knee joint in an 8-year-old haemophilia A patient with high inhibitor of 3450 Bethesda units (BU) is described. He had an infected compound fracture of the tibia and fibula. Surgery was successfully performed after extensive plasma exchange; administration of immunosuppressive agents such as cyclophosphamide, methylprednisolone, intravenous immunoglobulin and cyclosporine were combined with a loading dose of 100 units kg(-1) of factor VIII concentrate, followed by continuous infusion of 16 units kg(-1) h(-1) of factor VIII in the form of factor VIII concentrate and cryoprecipitate for 7 days and decreased to 8 units kg(-1) h(-1) in the form of cryoprecipitate for 19 more days. During the 1st to 7th post-operative days, the lowest factor VIII inhibitor was 18 BU and the factor VIII level ranged from < 1-2.1 IU dL(-1) . On the 9th and 13th post-operative day, although the inhibitor rose to 330 and 2700 BU, respectively, there was no serious bleeding. The suture was removed on the 21st post-operative day. The inhibitor spontaneously decreased to 550, 232 and 14 BU at 1, 7 and 10 months, respectively.
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