05 May 2014 : Case report
First successful allogeneic hematopoietic stem cell transplantation for a sickle cell disease patient in a low resource country (Nigeria): A case reportNosakhare BazuayeABCDEF, Benedict NwogohB, Douglas IkponmwenB, Omorodion IrowaB, Stanley OkugboB, Ime IsaB, Emmanuel IghodaroB, Yetunde-Isreal AinaB, Anthony OsaguonaB, Osagie IdemudiaB, Osaretin IyohaB, Okiroro IghoseweB, Dominic OsaghaeB, Christoph BucherABC
Ann Transplant 2014; 19:210-213
Background: Sickle cell disease (SCD) has a prevalence of 2–3% in Nigeria (population: over 150 million). We present our first allogeneic hematopoietic stem cell transplantation (HSCT) for a 7-year-old patient with severe sickle cell anemia and debilitating right-sided hemi-paraparesis.
Case Report: Conditioning was with (Reduced Intensity Conditioning (FLU/BU).[Fludarabine 160 mg/m2 (days –6 to –2) and Busulphan 16 mg/kg (4×25mg 6hly days –5 to –2) and Anti-thymocyte globulin(ATG)(ATGAM) total dose 500 mg (days –6 to –4)]. Graft versus Host Disease (GVHD) prophylaxis was with Cyclosporine A (2×50 mg daily) and Mycophenolate Mofetil (2×500 mg/day).Stem cell source was bone marrow harvested on the 28 September 2011 with 9.8×108 nucleated cells/kg in a total volume of 900 mL from his 14-year-old HLA-matched sibling (6/6).
Neutrophil and platelet engraftment was day +18 and +21, respectively. At day +70 full blood count was a total white blood cell count of 3100/µl, absolute Neutrophil count 1200/µl, Hemoglobin (Hb) 11.3 g/dl, Platelet 198,000/µl, Hemoglobin phenotype AA, and no acute or chronic GVHD. He is clinically stable with a Chimerism at 2 years post-HSCT of 95% and responding to physiotherapy.
Conclusions: We have successfully performed a stem cell transplanted in a 7-year-old Sickle Cell Anemia case. With the assistance of Government and improved Health Insurance Policy, we could make HSCT available as a cure for many Nigerians with both malignant and non-malignant disorders.
Keywords: sickle cell disease, Stem Cell Transplantation, low resource country
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