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Effect of transfusion therapy on arteriographic abnormalities and on
recurrence of stroke in sickle cell disease
MO Russell, HI Goldberg, A Hodson, HC Kim, J Halus, M Reivich and E Schwartz
Stroke is a relatively frequent and severe complication of sickle cell
disease. We performed cerebral arteriograms in 30 patients with sickle cell
disease to evaluate the cause of acute neurologic deficits and to assess
the effects of transfusion therapy given for a year or more after the acute
episode. Twenty-three patients with motor and speech deficits had multiple
abnormalities of major cerebral arteries. The internal carotid and anterior
and middle cerebral arteries showed stenosis and/or occlusion at their
common junction. Irregular luminal surfaces suggested that endothelial
damage and intimal hyperplasia were the basis of stroke. Prolonged
transfusion therapy nearly stopped progression of stenosis and markedly
decreased the irregularity of the luminal surfaces; in 4 untransfused
patients, the degree of stenosis doubled and the luminal abnormalities
persisted. Prior to transfusion, 90% of patients had recurrence of stroke.
With transfusion therapy, only 10% of patients had recurrence despite
persistent arterial abnormalities. Clinical recurrences per patient-month
decreased 75- fold. The patients tolerated prolonged transfusion therapy
well, despite progressive iron accumulation. Seven patients with smooth
abnormalities of a single artery, nonocclusive changes, or with normal
arteriograms did not receive transfusions. Only one of this group had
recurrence of symptoms.
Volume 63,
Issue 1,
pp. 162-169,
01/01/1984
Copyright © 1984 by The American Society of Hematology
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