Allogeneic Bone Marrow Transplantation from Haplomatched Sibling Donor in a Pediatric Patient with Sickle Cell Disease with Multiple Painful Crises: An Experience

Abstract

Bone marrow transplantation is the only cure for sickle cell disease but this curative therapy unfortunately lead to complication and several times death. Bone marrow transplantation indicated only for sickle cell patients with severe sickle cell disease who have complications including strokes, acute chest syndrome, recurrent pain crisis and exchange transfusions, nephropathy retinopathy, osteonecrosis of multiple joints and priapism. All sickle cell patients are not eligible for bone marrow transplantation due to associated toxicity [1]. Transplant is performed when benefit outweighs the risk. There has been much development and improvement in the bone marrow transplant since the first transplant done in 1984. This technique includes using chemotherapy for conditioning to remove the recipients cells and replacing it with donor cells free of sickling. Main problem limiting the transplant to all patients include getting a matched donor, risk associated with the procedure; including graft vs. host disease and cost. Hematopoietic stem cell transplant is safest when a matched donor is available. Even with this, there is still risk of graft rejection and chronic graft verses host disease. The unrelated cord blood stem cell transplant and haploidentical stem cell have been found to be less successful due to an increase in graft vs. host disease. Bone marrow transplantation has helped in improving the quality of life of the patient by eliminating the repeated hospital admission [2].