Postpartum factor VIII inhibitors. Report of two cases with special reference to the efficacy of various treatments.
Postpartum factor VIII inhibitors. Report of two cases with special reference to the efficacy of various treatments.
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产后因子 VIII 抑制剂。
DOI：
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发表时间：
1993
影响因子：
2.6
通讯作者：
K. Lechner
中科院分区：
文献类型：
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作者：
I. Hauser;H. Gisslinger;G. Locker;W. Elbl;P. Kyrle;I. Pabinger;K. Lechner
两例产后抑制因子VIII的描述。1例患者有严重出血倾向（髂腰肌出血、前臂出血）。病例1的最大抑制物滴度为800 BU/ml，病例2为120 BU/ml。在病例1中，通过使用人和猪凝血因子VIII浓缩物和活化凝血酶原复合物浓缩物（FEIBA，Immuno AG，维也纳，奥地利）治疗控制出血，而在病例2中未给予替代治疗。泼尼松（在两例病例中给予）和大剂量免疫球蛋白（在病例2中给予）无效。环磷酰胺（每天100毫克），结合小剂量的泼尼松导致完全消失的抑制剂和正常化因子VIII的情况下1。在另一名拒绝接受环磷酰胺治疗的患者中，抑制剂滴度在13个月内仍然很高。
Two cases of postpartum inhibitors to F VIII are described. One patient had a severe bleeding tendency (iliopsoas bleeding, forearm bleeding). The maximum inhibitor titer was 800 BU/ml in case 1 and 120 BU/ml in case 2. Bleeding was controlled by treatment with human and porcine factor VIII concentrate and with activated prothrombin complex concentrate (FEIBA, Immuno AG, Vienna, Austria) in case 1, while no replacement therapy was given in case 2. Prednisone (given in both cases) and high-dose immunoglobulin (given in case 2) was without effect. Cyclophosphamide (100 mg per day), combined with small doses of prednisone led to complete disappearance of the inhibitor and normalization of factor VIII in case 1. In the other patient who refused treatment with cyclophosphamide the inhibitor titer remained high over a period of 13 months.