阵发性睡眠性血红蛋白尿症（PNH）的治疗。
Management of paroxysmal nocturnal hemoglobinuria (PNH).
机构信息
Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar-es-Salaam, Tanzania.
University of Florence, Florence, Italy.
出版信息
J Clin Lab Anal. 2022 Dec;36(12):e24770. doi: 10.1002/jcla.24770. Epub 2022 Nov 15.
Abstract
PNH is a chronic hemolytic disorder due to an intrinsic red cell abnormality. There is no evidence that either prolonged administration of corticosteroids or chemotherapy are beneficial in PNH. On the other hand, patients can live with PNH for many years with supportive management. At the moment complement inhibitor therapy is indicated in most cases; and it is highly desirable that the current financial barriers to this therapy be overcome.
摘要
PNH 是一种由于内在红细胞异常引起的慢性溶血性疾病。没有证据表明长期使用皮质类固醇或化疗对 PNH 有益。另一方面，患者可以通过支持性治疗在 PNH 中存活多年。目前，大多数情况下都需要补体抑制剂治疗；非常希望克服目前治疗这种疾病的财务障碍。
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