Reversible posterior leukoencephalopathy syndrome in sickle-cell anemia

镰状细胞贫血症中的可逆性后部白质脑病综合征

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Abstract

A 10-year-old African American girl with sickle-cell anemia developed headaches and seizures associated with hypertension during hospitalization for a pulmonary abscess. Hypertension developed after multiple transfusions, associated with abnormally high hematocrit and headache. Magnetic resonance imaging was consistent with posterior leukoencephalopathy. Neurologic signs, hypertension, and high hematocrit resolved after erythrocytapheresis. Magnetic resonance imaging, 1 month after the episode, produced normal results. Because reversible posterior leukoencephalopathy syndrome was only described in sickle-cell anemia during severe acute chest syndrome, this report documents that milder illness can be associated with reversible posterior leukoencephalopathy syndrome in sickle-cell anemia, and also highlights subtle signs that may herald serious neurologic events in high-risk patients. Examination of the pathophysiology of reversible posterior leukoencephalopathy syndrome in the context of sickle-cell anemia suggests that patients with sickle-cell anemia and subtle neurologic signs should be treated with high vigilance.

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