2605

BONE MARROW TRANSPLANTATION FOR ß-THALASSEMIA MAJOR BY HLA MISMATCHED MOTHER

Li CF¹, Zhang YM¹, Wu XD¹, He YL¹, Sun J¹, Chen SJ¹, Li CK² and Tsang KS²

¹Nanfang Hospital, Guangzhou, china;

²The Chinese University of Hong Kong, Hong Kong, China.

Objective: To study the possibility of Bone Marrow Transplantation (BMT) for β-thalassemia Major using HLA (human leukocyte antigen) Mismatch mother donor.

Methods: The patient is a boy, six and half years old. When he is six months old, he was diagnosed as β-thalassemia Major. Gene-type: 41-42/41-42. Since then, he must receive blood transfusion monthly for life term and iron chelation therapy irregularly. The liver and spleen are detected at 5cm and 1cm below the costal margin before BMT, respectively. His mother, HLA mismatched (5/6), was selected as donor. An improved regimen was used. It included more intensive immunosuppress, as well as, a normal feed-back mechanism was used. This regimen included TBI(total body irradiation), Bu (Busulfan), Cy(cyclophosphamide) and ATG (Antithymocyte globulin). Prophylaxis of GVHD (graft versus host disease) include CsA(Cyclopsprin A) and Methyeprednisolone.

Results: Complete donor chimerism was detected with FISH(fluorescence in situ hybridization) on day +20, +43,+147. I degree of a GVHD was seen. Now, 18 months post-transplantation, Hb is above 100g/L and infuse-free. As we know, this is the first successful case of BMT for Ⅲ°thalassemia using HLA mismatched parent donor.

Conclusion: Bone Marrow Transplantation for β-thalassemia Major using one HLA mismatched mother donor is possible.