Age 3.5 years, β-thalassemia major
Contributed By: Dr. Imtenan Sharif
Sahir Fatima from Multan was diagnosed with β-thalassemia major on 25th Feb, 2001. She received her first blood transfusion at the age of 3.5 years. Since then, she was transfused blood on monthly basis. She reported at AFBMTC on 12th April, 2014 with 8/8 HLA matched sibling.
Sahir was 15 years of age and had approximately received 150 RCC transfusions when she reported at AFBMTC. On examination liver was palpable 3 cm below right costal margin with rest of the examination being unremarkable.
Her liver biopsy was performed which revealed a Knodell Score as follows: Hepatic Activity Index 2/18 and Fibrosis 1/6. During pretransplant assessment, Sahir’s anti HBc antibody and anti HCV antibody came up positive. However, PCR for HCV RNA and HBV DNA were negative. There was no ABO mismatch.
Conditioning for transplant was given with long protocol of Flu150 BuIV Cy160 Thiotepa10 and ATG10. She tolerated conditioning well. Bone marrow was harvested for her donor sister) on 4th Sep, 2015 and the harvest yielded a Total Nucleated Cell dose of 5.13 x108/kg and a CD34 count of 8.19 x106/kg.
The patient received cyclosporine and methotrexate for graft versus host disease prophylaxis. She developed mild hematuria on D+10 which was managed conservatively and settled by D+14. Neutrophil engraftment was achieved on D+15 and platelet engraftment a little later on D+27.
Sahir’s immunosuppression was tapered off and stopped after 1 year of transplant and she was revaccinated as per protocol. Her donor chimerism remained more 90% and lymphocyte subset analysis demonstrated good immune reconstitution. Sahir is a Survivor. She is now studying in college and enjoys good quality of life with her family and friends.