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凝血因子VIII抑制剂:检测与治疗

Inhibitors of factor VIII: detection and treatment.

作者信息

Brettler D B

机构信息

University of Massachusetts Medical School, Worcester 01605.

出版信息

Southeast Asian J Trop Med Public Health. 1993;24 Suppl 1:21-5.

PMID:7886576
Abstract

Alloantibodies occurring in hemophiliacs is a side effect or repeated treatment and represents a severe complication. The induction of immune tolerance using one of the lower dose regimens should be attempted as soon as it is feasible as regimens started soon after the inhibitor appears may have greater success in inducing tolerance. If the hemophiliac with inhibitor hemorrhages, PCC for aPCC should be the first line of therapy since these concentrates can be given in the home setting. If the hemorrhage is severe and the anti-porcine inhibitor titer is low, the patient should be infused in a clinic or hospital setting with porcine factor VIII using increasing doses to achieve a circulating factor VIII level. Entry into clinical trials, such as those using rFVIIa should be encouraged.

摘要

血友病患者中出现的同种抗体是重复治疗的副作用,也是一种严重并发症。一旦可行,应尽快尝试使用较低剂量方案之一诱导免疫耐受,因为在抑制剂出现后不久开始的方案在诱导耐受方面可能更成功。如果有抑制剂的血友病患者出血,应首选凝血酶原复合物浓缩剂(PCC)或活化凝血酶原复合物浓缩剂(aPCC)进行治疗,因为这些浓缩剂可以在家中使用。如果出血严重且抗猪抑制剂滴度较低,患者应在诊所或医院环境中输注猪源凝血因子VIII,逐步增加剂量以达到凝血因子VIII的循环水平。应鼓励患者参加临床试验,如使用重组凝血因子VIIa(rFVIIa)的试验。

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