PROGRESSIVE CEREBRAL ARTERIAL DISEASE DESPITE A SUCCESSFUL HEMATOPOIETIC STEM CELL TRANSPLANT FOR SICKLE CELL ANEMIA

Sickle Cell disease often results in cerebral arterial disease including stroke and moyamoya syndrome. Hematopeoietic stem cell transpant (HSCT) is the definitive cure for sickle cell disease, and often slows or stops the progression of the related cerebral arterial disease. A case is presented of a patient whose cerebral arterial disease progressed despite successful stem cell transplant. This project will describe this patient’s clinical course and briefly explore outcome expectations post HSCT in patients with existing moyamoya syndrome. Methods Medical records chart review of one case. Results A 9 year old female experienced cerebral arterial complications of sickle cell disease resulting in stroke. She was then started on monthly transfusions to prevent further strokes. During workup for hematopoietic stem cell transplant (HSCT), she was found to have progressive cerebral arteriopathy (Moyamoya syndrome). She then underwent successful transplant using unrelated cord blood. Shortly following HSCT, she experienced relatively common complications including chronic graft versus host disease, thrombocytopenia, anemia, Epstein-Barr disease reactivation, and several bacterial and fungal infections. These conditions resolved with treatment. HSCT was successful in completely resolving the sickle cell disease. Despite this, she experienced seizures, progression of moyamoya syndrome and a new cerebral infarct. Revascularization surgery has been recommended. Conclusions In some cases, cerebral arterial disease may progress despite successful hematopoietic stem cell transplantation. These cases may suggest a need for further research into the outcomes of sickle cell patients with existing moyamoya syndrome prior to HSCT. Purpose (a): Sickle Cell disease often results in cerebral arterial disease including stroke and moyamoya syndrome. Hematopeoietic stem cell transpant (HSCT) is the definitive cure for sickle cell disease, and often slows or stops the progression of the related cerebral arterial disease. A case is presented of a patient whose cerebral arterial disease progressed despite successful stem cell transplant. This project will describe this patient’s clinical couse and briefly explore outcome expectations post HSCT in patients with existing moyamoya syndrome. Methods (b): A medical chart review of one case was conducted. Results (c): A 9 year old female experienced cerebral arterial complications of sickle cell disease resulting in stroke. She was then started on monthly transfusions to prevent further strokes. During workup for hematopoietic stem cell transplant (HSCT), she was found to have progressive cerebral arteriopathy (Moyamoya syndrome). She then underwent successful transplant using unrelated cord blood. Shortly following HSCT, she experienced relatively common complications including chronic graft versus host disease, thrombocytopenia, anemia, Epstein-Barr disease reactivation, and several bacterial and fungal infections. These conditions resolved with treatment. HSCT was successful in completely resolving the sickle cell disease. Despite this, she experienced seizures, progression of moyamoya syndrome and a new cerebral infarct. Revascularization surgery has been recommended. Conclusions (d): In some cases, cerebral arterial disease may progress despite successful hematopoietic stem cell transplantation. These cases may suggest a need for further research into the outcomes of sickle cell patients with existing moyamoya syndrome prior to HSCT.