NEUROLOGIC COMPLICATIONS FOLLOWING BONE-MARROW TRANSPLANTATION FOR SICKLE-CELL DISEASE

A boy with sickle cell anemia underwent bone marrow transplantation (B MT), He was normal on neurological examination, but had radiologic evi dence of an old left frontal lobe infarct, multiple cerebral vascular stenoses and moyamoya collaterals, After BMT he developed seizures wit h extension of the infarct and subarachnoid hemorrhage, One year later angiography revealed worsening stenosis of the M1 segments of both mi ddle cerebral arteries, At that time an increase in von Willebrand's f actor with decreased large molecular weight multimers (LvWF) was obser ved, We speculate that LvWF dependent, shear-induced platelet aggregat ion, together with endothelial damage may have contributed to the deve lopment of neurologic complications in this patient.