How I treat acute strokes and long-term management in sickle cell disease.
Blood. 2015 Mar 30;
Authors: Kassim AA, Galadanci NA, Pruthi S, DeBaun MR
Neurological complications are a major cause of morbidity and mortality in sickle cell disease. In children with sickle cell anemia, routine use of transcranial Doppler screening coupled with regular blood transfusion therapy has decreased the prevalence of overt stroke from approximately 11% to 1%. Limited evidence is available to guide acute and chronic management of individuals with sickle cell disease and strokes. Current management strategies are based primarily on single arm clinical trials and observational studies, coupled with principles of neurology and hematology. Initial management of a focal neurological deficit includes evaluation by a multi-disciplinary team (a hematologist, neurologist, neuroradiologist and transfusion medicine specialist); prompt neuro-imaging, and an initial blood transfusion (simple followed immediately by an exchange transfusion or only exchange transfusion), is recommended if the hemoglobin is > 4 gm/dL and < 10 gm/dL. Standard therapy for secondary prevention of strokes and silent cerebral infarcts includes regular blood transfusion therapy and in selected cases, hematopoietic stem cell transplantation. A critical component of the medical care following an infarct is cognitive and physical rehabilitation. We will discuss our strategy of acute and long-term management of strokes in sickle cell disease.
PMID: 25824688 [PubMed - as supplied by publisher]