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Acute Graft Versus Host Disease Following Sibling Donor Transplantation for Thalassemia Major
This is a retrospective analysis of 321 patients who underwent allogeneic SCT for Thalassemia Major at our centre between Jan 1991 and Dec 2011. This included 205 males and 116 females with a median age of 7 years (range: 2 - 24). Patients who expired prior to 2 weeks or had primary graft rejection were excluded from this analysis. 6.9% of patients were in Lucarelli Class I, 36.4% in Class II, and 56.7% in Class III. Donors included matched sibling (n = 299) or other family donors (n = 22). Conditioning regimen was predominantly Busulfan based (n = 274) while 47 patients received treosulfan based conditioning. Graft source was mainly bone marrow (n = 286) while GVHD prophylaxis mainly was Cyclosporine with short course methotrexate (n = 301).
Acute GVHD (Grade I – IV) occurred in 125 patients (38.9%), grade II-IV in 28% and grade IV in 5.3%. Donor age (p = 0.062), type of conditioning regimen (p = 0.07), number of doses of methotrexate administered (p = 0.05), presence of veno-occlusive disease (VOD) [p = 0.016] and time to neutrophil engraftment (p = 0.027) were found to be significant risk factors for acute GVHD on a univariate analysis but only VOD (p = 0.017) and donor age (p = 0.044) remained significant on multivariate analysis. Resolution of GVHD was seen in 90.4% while 9.6% died either due to GVHD or infection. The 3 year overall survival was 80% ± 2.3% in patients with GVHD compared to 81.1% ± 2.9% in patients without GVHD (p = 0.422).
Acute GVHD is seen in 39% of patients undergoing SCT for thalassemia major but it does not have a significant impact on overall survival.