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[活化蛋白C抵抗所致易栓症诊断的初步经验]

[Initial experience in the diagnosis of thrombophilia due to activated protein C resistance].

作者信息

Barkagan Z S, Gervaziev V B, Tsyvkina L P, Mamaev A N, Karpenko A A, Tseĭmak I Ia

出版信息

Ter Arkh. 1997;69(2):35-7.

PMID:9173574
Abstract

The authors describe the first in Russia cases of thrombophilia caused by factor Va resistance to activated protein C. This abnormality was diagnosed in 6 of 25 patients (24%) with recurrent early thrombosis. The diagnosis was conducted according to a modified method implying the addition of protac (activator of protein C) to normal platelet poor plasma (PPP) free of factor V containing 100% of protein C. Protac dose was adjusted to increase the activated partial thromboplastin time (APTT) 2.4-2.8-fold in mixing PPP of the examinee with plasma containing activated protein C. The indices were estimated indicative of insufficient prolongation of APTT in response to activated protein C. Of 6 patients, 2 had apparent and 4 strong resistance to activated protein C. Treatment policy in this variant of thrombophilia is discussed.

摘要

作者描述了俄罗斯首例因因子Va对活化蛋白C抵抗而导致的血栓形成倾向病例。在25例复发性早期血栓形成患者中,有6例(24%)诊断出这种异常情况。诊断是根据一种改良方法进行的,该方法是将凝血酶原激活物(蛋白C激活剂)添加到不含因子V且含有100%蛋白C的正常少血小板血浆(PPP)中。调整凝血酶原激活物的剂量,以使受检者的PPP与含有活化蛋白C的血浆混合后,活化部分凝血活酶时间(APTT)延长2.4至2.8倍。评估了一些指标,以表明APTT对活化蛋白C反应的延长不足。在这6例患者中,2例对活化蛋白C有明显抵抗,4例有强烈抵抗。文中讨论了这种血栓形成倾向变体的治疗策略。

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