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Introduction: Hematopoietic Stem Cell transplantation (HSCT) is the only curative therapy for some malignant and non-malignant diseases including Sickle cell disease(SCD).The world celebrated the one millionth HSCT in December 2012 with only 2% of these HSCTperformed in East Mediterranean /Africa. Nigeria performed her first successful HSCT in 2011 and since then only a total of 3 HSCT have been performed.Activities: The stem cell transplant centre at the University of Benin Teaching Hospital Edo State commenced in 2011 and has performed 3 HSCT.Indications for HSCT were Sickle cell disease complicated with Cerebrovascular accident, for the first patient and refractory vaso-occlusive crises for the other two patients.Patients were all males with ages of 7,12 and 15years.Donors were matched siblings with no major blood group mismatch and with ages of 14,19 and 21years respectively.Conditioning regimen for all patients was reduced intensity FLU/BU(Fludarabin 160mg/m2 days -6 to -2,Busulphan 16mg/kg days -5 to -2) and Anti-thymocytes globulin(ATG (ATGAM 22.73mg/kg).However Prophylaxis for GVHD was with Cyclosporin A and MycophylnolateMefotil (MMF).All patients had un-manipulated bone marrow stem cells. A second HSCT was performed for the second patient who had primary rejection with a conditioning regimen of BU/CY Busulphan 16mg/kg/Cyclophosphamide 100mg/kg pre-transplant and 100mg/kg atday +3 and +4 post- transplantResults: Overall survival is 100% and 67.7% disease free survival. Neutrophil engraftments for the two successful HSCT were both at day +18 while platelets were at days +21 and +22respectively.Chimerism at one year post transplant was 95 and 96% for the first and third patient respectively.No recorded acute or chronic graft versus host disease but there was a single report of femoral catheter infection with Pseudomonas that responded to antibiotics.The second HSCT had a primary rejection for both the first and second HSCT but developed a high persistent fetal hemoglobin of 29.5% .He has remained clinically stable 2 years post- transplant with few insignificant crises.Conclusion: A single Centre report of HSCT activities for Sickle cell disease with no mortality. There is need to perform more transplantations to improve the activities and experience in the Centre. Also with the huge population of Nigeria, there is the need to have more transplant Centres to meet the needs of Nigerians who will require HSCT.Keywords: Hematopoietic stem cell transplantation, sickle cell disease, single centre |
