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Intracranial Hemorrhage in Patients With Sickle Cell Disease

Am J Dis Child. 1986;140(6):505-506. doi:10.1001/archpedi.1986.02140200015011.
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Sir.—In their recent article van Hoff et al1 failed to mention a special group of patients with sickle cell disease who have a characteristic syndrome of post-transfusion hypertension, seizures, and intracranial hemorrhage. We2 reported three children (aged 6, 10, and 12 years, respectively) with this syndrome. Two children developed the three components of the syndrome (hypertension, seizure, and intracranial hemorrhage); a spinal tap was not done on the third child. Royal and Seeler3 reported two children (aged 8 and 14 years, respectively) with the syndrome; one child had intracranial hemorrhage while the other child had normal cerebrospinal fluid. The only adult patient reported with this syndrome had hypertension and seizures but no intracranial hemorrhage.4 A similar syndrome was reported by Wasi et al5 in patients with thalassemia major.

The pathogenesis is not known; however, it might be multifactorial. Release of a vasopressor substance from


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