Elsevier

The Journal of Pediatrics

Volume 96, Issue 2, February 1980, Pages 205-208
The Journal of Pediatrics

Original article
Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents

https://doi.org/10.1016/S0022-3476(80)80803-1Get rights and content

Since 1974 we have entered 12 children with sickle cell disease and strokes on a transfusion protocol to maintain hemoglobin S<20%. Serial arteriography, EEGs, brain or CT scans, and neuropsychologic testing were also obtained. Transfusion has been stopped in ten patients after one to two years. Seven of these ten patients have had second strokes five weeks to 11 months after cessation of transfusion (median three months). Arteriography was normal at the time of the initial stroke in two patients; one of these had a second stroke. Arteriograms did not improve during transfusion therapy. EEGs and brain and CT scans were occasionally useful at the time of the initial stroke but were of little value in following these patients. Neuropsychologic testing indicated severe impairment of sensory-motor and cognitive processes at the time of the initial stroke and was useful in following improvement or deterioration and in designing remedial education programs. We conclude that short-term transfusion therapy will not prevent second strokes once transfusion is stopped and that arteriography is of limited value in these patients.

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Cited by (146)

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    In 10 children with SCA and previous stroke, when pRBC transfusions to maintain HbS < 20% were stopped after 1 or 2 years of treatment, the recurrence rate was similar to that found in non-transfused patients with previous stroke. The second stroke occurred 5 weeks to 11 months after stopping transfusions (median 3 months) in 7/10 patients.18( C) Similar results were found in another 10 children with SCA and previous stroke (median 9½ years, range 5–12 years) after stopping pRBC transfusions (HbS < 30%): high recurrence rate within 12 months.

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    Regular transfusion of sickle-negative blood to maintain a trough hemoglobin S less than 30% of total hemoglobin is the most commonly used therapy for the secondary prevention of stroke. Recurrence rates after first ischemic stroke are as high as 67%, but decrease to 2–4 per 100 person-years with regular transfusions of sickle-negative blood (Wilimas et al., 1980; Wang et al., 1991; Balkaran et al., 1992). Transfusion is the most frequently used therapy in children, but there is some evidence that hydroxyurea also reduces stroke recurrence.

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    Given the multiple options of therapy for this patient, a family meeting was deemed critical to ensure a full understanding of the trade-offs for a secondary stroke prevention option. The possibility of stopping transfusion therapy was mentioned with the recognition that this would have the highest rate of recurrence.88,89 We also discussed the evidence that regular blood transfusion is not the definitive therapy, and we expect 45% of patients will have a new cerebral infarct7 over a course of 5.5 years.

  • Stroke With Transfusions Changing to Hydroxyurea (SWiTCH)

    2012, Blood
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    An effective therapeutic target for transfusions is 30% HbS, associated with a 14% to 23% incidence of secondary stroke and event rate of 2.2-6.4 recurrent strokes per 100 patient-years.3,7,8 Unfortunately, chronic transfusions are administered indefinitely, because of the high stroke recurrence rate after discontinuation of short-term9 or long-term10 transfusions. Consequently, young patients with SCA and stroke remain on lifelong chronic transfusions with almost no available alternatives, and develop transfusion-associated problems including infections, erythrocyte auto/alloimmunization, iron overload requiring chelation therapy, and reduced expected number of quality-adjusted life years.11-15

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Supported in part by CA 23944 and CA 21765 from the National Cancer Institute and by ALSAC.

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