Age 4 years, β-thalassemia major
Contributed By: Dr. Imtenan Sharif
Hadia is daughter of an officer serving in the Pakistan Army. She was diagnosed as case of β-thalassemia major at the age of 6 months. The patient was 4 years old when she first reported at our center. She was 8/8 HLA matched with her younger sister Anoosha. At the time of transplant, Hadia was 4 years and 2 months old while her donor Anoosha was 9 months of age.
Hadia underwent transplant on 25 Jan 2008. Her pre-transplant conditioning consisted of short protocol consisting of Busulfan and Cyclophosphamide. Stem cells were collected by bone marrow harvest. Cyclosporine was given for graft versus host disease (GVHD) prophylaxis. The patient had prompt engraftment with neutrophil recovery on day+15. There were no significant transplant related complications. Post-transplant, STRs for donor chimerism remained more than
90% and lymphocyte subset analysis showed good immune reconstitution. Immunosuppression was tapered off and stopped after 1 year of transplant and vaccination was done as per protocol.
The patient required a few sessions of phlebotomies for high serum ferritin levels (3619 ng/ml). There is no sign of GVHD with normal growth pattern. Hadia is now 13 years old and is in 7th class while her donor Anoosha is of 10 years of age studying in class 4. Anoosha is the youngest bone marrow donor for a sibling at our center.