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A modified transfusion program for prevention of stroke in sickle cell
disease
AR Cohen, MB Martin, JH Silber, HC Kim, K Ohene-Frempong and E Schwartz
Department of Clinical Laboratories, Children's Hospital of Philadelphia,
PA 19104.
Regular red blood cell transfusions reduce the rate of recurrent cerebral
infarction in sickle cell disease but lead to accumulation of excessive
iron. We studied the effect on the prevention of recurrent stroke and the
volume of blood transfused of a modified transfusion program in which the
pretransfusion percentage of hemoglobin S (HbS) was maintained at 50%,
rather than the conventional 30%. Fifteen patients with sickle cell disease
and cerebral infarction who had been free of recurrent stroke for at least
4 years during which the pretransfusion HbS was maintained below 30% were
assigned to a transfusion program in which the HbS was allowed to increase
to 50%. Transfusion regimens included simple transfusion and manual and
automated partial exchange transfusion. The duration of follow-up was 14 to
130 months with a median duration of 84 months. None of the 15 patients had
a recurrent cerebral infarction during 1,023 patient- months in which the
target pretransfusion HbS was 50%. Analysis of this finding, using a
binomial distribution, indicates that there is less than a 5% chance that
the risk per patient of recurrent stroke in the first year of the modified
transfusion program is greater than 18%. One 23-year-old patient had a
fatal intraventricular hemorrhage when the HbS was 30% and a 21-year-old
patient had a fatal subarachnoid hemorrhage in the 40th week of pregnancy
when the HbS was 29%. Blood requirements with simple transfusions decreased
by 17% to 48% (mean 31%) when the target pretransfusion HbS level was
increased from 30% to 50% (P less than .001). Manual or automated partial
exchange transfusions and a target HbS level of 50% in eight patients
reduced blood requirements by 33% to 99% (mean 67%) in comparison with
simple transfusion and a target HbS level of 30% (P less than .001). This
study offers evidence that a target pretransfusion HbS level of 50% affords
a continuing high rate of protection against recurrent cerebral infarction
in sickle cell disease after 4 years of a conventional transfusion program.
Increasing the target HbS level from 30% to 50% provides a major reduction
in blood requirements and lowers the rate of iron accumulation.
Volume 79,
Issue 7,
pp. 1657-1661,
04/01/1992
Copyright © 1992 by The American Society of Hematology
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