IMPROVEMENT OF CEREBRAL VASCULAR DISEASE IN SICKLE CELL ANEMIA FOLLOWING TRANSFUSIONS

Abstract

Cerebrovascular accidents (CVA) are a major clinical problem in sickle cell disease. We have studied four affected children soon after CVA and one year later to evaluate the effect of chronic transfusion therapy on arterial abnormalities. Percutaneous femoral-cerebral angiography was performed after careful preparation of patients by partial exchange or repeated transfusions to achieve a normal hemoglobin concentration and reduction of Hb S to about 20%. There were no complications of angiography. When possible, the right and left carotid and basilar systems were visualized and cerebral blood flow was measured with ¹³³Xe. Major cerebral artery disease, often more extensive than was clinically evident, was found in all cases. Three of the four children were transfused for one year. Two showed complete resolution of occlusive disease. In a third child in whom transfusions were not started until after a second CVA, some affected vessels had improved after a year, but others showed no change or chronic narrowing. Cerebral blood flow (133xe), initially decreased in this child, was normal on the second study. A fourth child not transfused between studies showed severe occlusive disease with progression. These data suggest cerebrovascular damage in sickle cell disease may be reversible with long-term transfusion therapy.

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