Combined congenital deficiency of factor V and factor VIII. Report of a further case with some considerations on the hetereditary transmission of this disorder.
Combined congenital deficiency of factor V and factor VIII. Report of a further case with some considerations on the hetereditary transmission of this disorder.
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因子 V 和因子 VIII 联合先天性缺乏。
DOI：
10.1159/000208020
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发表时间：
1976
影响因子：
2.4
通讯作者：
A. Galletti
中科院分区：
文献类型：
--
作者：
A. Girolami;G. Gastaldi;G. Patrassi;A. Galletti
合并因子V和因子VIII缺乏症的患者。出血表现轻微。主要实验室特征为部分凝血活酶时间延长，通过添加吸附正常血浆进行校正，但未通过添加正常血清、血友病A血浆或另一名因子V和因子VIII联合缺乏患者的血浆进行校正。TGT也明显异常，并通过添加吸附的正常血浆而不是通过添加正常血清进行校正。凝血酶原消耗轻度缺陷。凝血酶原时间略有延长。凝血因子VIII为正常值的12%，凝血因子V为正常值的55%。因子VIII相关抗原正常。先证者的父亲和一个姐姐表现出轻度的因子V缺乏，但正常的因子VIII活性和抗原。父母不是血亲。本文提出了两组凝血因子V和VIII联合缺乏症的初步分类。遗传传递的两种类型的缺陷进行了讨论。
A patient with combined factor V and factor VIII deficiency is presented. The bleeding manifestations were mild. The main laboratory feature was a prolonged partial thromboplastin time which was corrected by the addition of adsorbed normal plasma but not by the addition of normal serum, hemophilia A plasma or plasma of another patient with combined factor V and factor VIII deficiency. TGT was also clearly abnormal and was corrected by the addition of adsorbed normal plasma but not by the addition of normal serum. Prothrombin consumption was mildly defective. The prothrombin time was slightly prolonged. Facotr VIII was 12% and factor V 55% of normal. Factor-VIII-associated antigen was normal. The father and a sister of the propositus revealed mild factor V deficiency but normal factor VIII activity and antigen. The parents were not consanguineous. A tentative classification of combined deficiency of factors V and VIII in two groups is proposed. The hereditary transmission of the two types of deficiencies is discussed.