Haemophilia due to factor VIII inhibitors in a patient suffering from an autoimmune disease: Treatment with intravenous immunoglobulin
Haemophilia due to factor VIII inhibitors in a patient suffering from an autoimmune disease: Treatment with intravenous immunoglobulin
复制标题
患有自身免疫性疾病的患者因因子 VIII 抑制剂导致血友病：静脉注射免疫球蛋白治疗
DOI：
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发表时间：
1984
期刊：
影响因子：
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通讯作者：
P. Imbach
中科院分区：
文献类型：
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作者：
A. Gianella;A. Hirt;A. Lüthy;H. Wagner;P. Imbach
摘要本文描述了一例因凝血因子VIII抑制剂引起的血友病病例，该病例发生在一名患有自身免疫性疾病的13岁男孩身上。患者有抗第八因子的自身抗体。血友病被长春新碱和类固醇控制，但由于副作用而不得不停止这种方案，因此血友病复发。静脉注射免疫球蛋白(Ig G)治疗产生因子缓慢上升，而因子抑制剂消失。虽然因子VIII活性仅在几个月内升高，并且因子VIII抑制物再次出现，但免疫球蛋白治疗仍在继续，患者仍然没有临床症状。并对免疫球蛋白治疗的作用机制进行了讨论。
SummaryThis paper describes a case of haemophilia due to factor VIII inhibitors occurring in a 13-year-old boy suffering from an autoimmune disease. The patient had autoantibodies to factor VIII. The haemophilia was controlled by vincristine and steroids, but this regimen had to be discontinued because of side effects, whereupon the haemophilia recurred. Treatment with intravenous immunoglobulin (IgG i.v.) produced a slow rise in factor VIII, and the factor VIII inhibitors disappeared. Although factor VIII activity was raised for only a few months and factor VIII inhibitors reappeared, immunoglobulin treatment was continued and the patient remained remained free of clinical symptoms. The mechanism of action of treatment with IgG is discussed.
两种不同类型的人类抗体使因子 VIII 凝血活性失活。
影响因子：
20.3
作者：
Gawryl,MS;Hoyer,LW
通讯作者：
Hoyer,LW