A modified transfusion program for prevention of stroke in sickle cell disease

Abstract

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.

Management Of Cerebral Vasculopathy In Children With Sickle Cell Anaemia

“…Functional neurological de®cits persist and become more obvious as the child matures (Hariman et al, 1991;Craft et al, 1993;Bernaudin et al, 1998;Wang et al, 1998a). As young adults, they often die of massive intracranial haemorrhage, adult respiratory distress syndrome (ARDS) or complications of transfusion-induced haemochromatosis (Powars et al, 1990a;Cohen et al, 1992;Miller et al, 1992;Platt et al, 1994).…”

Red blood cell exchange in patients with sickle cell disease—indications and management: a review and consensus report by the therapeutic apheresis subsection of the AABB

“…5,17 The optimal duration of treatment is unknown, 18 but many centers continue transfusion treatment indefinitely for patients who have had a previous stroke. 19 Chronic transfusion programs also have been used to prevent recurrent acute chest syndrome and vasoocclusive pain crises. 11 Some institutions perform RCEs for the prevention and treatment of priapism.…”

“…17,58 Some small studies suggest that it may be possible, if patients have been stable neurologically for at least 4 years after the first stroke, to maintain them at a goal HbS level of less than 50% without significantly increasing their risk. 7,19,59 Currently there is no consensus on the transfusion strategy that should be used for patients with SCD undergoing surgery. 23 Evidence-based guidelines recommend preoperative transfusion to bring Hb level to 10 g/dL.…”

The Role of Red Blood Cell Exchange Transfusion in the Treatment and Prevention of Complications of Sickle Cell Disease

“…After a stroke, without preventive therapy, recurrence rates from 46% to 90% have been reported (7-9). However, use of chronic transfusion programs have been shown effectively to reduce recurrence rates to <15% (8,(10)(11)(12)(13)(14). Usually, every 3-4 weeks, patients receive either simple or exchange transfusions so that HbS is kept <30%, and the total Hb concentration remains about 10 g/dl.…”