Congenital Combined Deficiency of Factor V and Factor VIII
Congenital Combined Deficiency of Factor V and Factor VIII
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先天性因子 V 和因子 VIII 联合缺乏症
DOI：
10.1055/s-0038-1651357
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发表时间：
1969
影响因子：
6.7
通讯作者：
O. Katsumi
中科院分区：
文献类型：
--
作者：
H. Saito;M. Shioya;K. Koie;T. Kamiya;O. Katsumi
摘要报告1例先天性V、VIII因子合并缺乏。患者为一名9岁男孩，自2岁起有鼻出血、血肿和拔牙后出血史；血浆中因子V和因子VIII的水平分别为正常水平的16%和8%。他的家族中没有人缺乏这两种因素。对1例a型血友病患者正常新鲜血浆和全血输血的效果进行了研究。前者与经典血友病相似，后者包括因子V活性的立即增加和因子VIII活性的延迟增加，尽管供者血液中几乎没有因子VIII活性。
Summary A case of congenital combined deficiency of factor V and factor VIII was reported. The patient, a 9 year old boy, gave a history of epistaxis, hematomas, and of hemorrhages following dental extraction since the age of 2 ; plasma levels of factor V and factor VIII were found to be 16% and 8% of normal, respectively. No one in his family had a deficiency of either factor. The effects of transfusion of normal fresh plasma and whole blood from a patient with hemophilia A were studied. While the former were similar to the pattern as seen in classical hemophilia, the latter consisted of an immediate increase of factor V activity and a delayed increase of factor VIII activity, despite the fact that factor VIII activity was almost absent from the donor’s blood.