Nosakhare Bazuaye1, Benedict Nwogoh1, Douglas Ikponmwen1, Omorodion Irowa2, Stanley Okugbo3, Ime Isa4, Emmanuel Ighodaro5, Yetunde-Isreal Aina6, Anthony Osaguona7, Osagie Idemudia8, Osaretin Iyoha9, Okiroro Ighosewe10, Dominic Osaghae11, Christoph Bucher12. 1. Department of Hematology, University of Benin, Teaching Hospital, Benin, Nigeria. 2. Department of Surgery, University of Benin, Teaching Hospital, Benin, Nigeria. 3. Department of Cardiothoracic, University of Benin, Teaching Hospital, Benin, Nigeria. 4. Department of Anaesthesia, University of Benin, Teaching Hospital, Benin, Nigeria. 5. Department of Radiology, University of Benin, Teaching Hospital, Benin, Nigeria. 6. Department of Child Health, University of Benin, Teaching Hospital, Benin, Nigeria. 7. Department of Maxillo-Facial, University of Benin, Teaching Hospital, Benin, Nigeria. 8. Department of Chemical Pathology, University of Benin, Teaching Hospital, Benin, Nigeria. 9. Department of Medical Microbiology, University of Benin, Teaching Hospital, Benin, Nigeria. 10. Department of Child Health, Delta State University, Teaching Hospital, Abraka, Nigeria. 11. Department of Child Health, Sickle Cell Centre, Benin, Nigeria. 12. Department of Hematology, Basel University, Teaching Hospital, Basel, Switzerland.
Abstract
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 (4×25 mg 6 hly 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.
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 (4×25 mg 6 hly 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.