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家族性狼疮抗凝物

Familial lupus anticoagulant.

作者信息

Tănăseanu C, Tănăseanu S

机构信息

Emergency Hospital, SF. Pantelimon, Department of Internal Medicine, Bucharest, Romania.

出版信息

Roum Arch Microbiol Immunol. 1999 Jul-Dec;58(3-4):259-65.

PMID:11845463
Abstract

The antiphospholipid antibody syndrome (APS) is defined by widespread arterial and venous thromboses associated with elevated plasma levels of antiphospholipid antibodies (APLA). The primary antiphospholipid antibody syndrome (PAPS) appear to be a fairly homogeneous disease, and HLA, family and other studies provide new insights into this cause of thrombosis and vascular disease. We describe two patients with PAPS (lupus anticoagulant positive), whose family members were analyzed for clinical and laboratory abnormalities associated with APS. Familial screening seems to be important, in order to prevent the thrombotic events. Low dose aspirin is the first line treatment in asymptomatic subjects with APLA, previous or present thrombosis requiring long-term, possibly life-long anticoagulation.

摘要

抗磷脂抗体综合征(APS)的定义是广泛的动静脉血栓形成,伴有血浆抗磷脂抗体(APLA)水平升高。原发性抗磷脂抗体综合征(PAPS)似乎是一种相当同质的疾病,而HLA、家族及其他研究为这种血栓形成和血管疾病的病因提供了新的见解。我们描述了两名患有PAPS(狼疮抗凝物阳性)的患者,对其家庭成员进行了与APS相关的临床和实验室异常分析。为预防血栓形成事件,家族筛查似乎很重要。对于有APLA的无症状受试者,低剂量阿司匹林是一线治疗方法,既往或目前有血栓形成者则需要长期、可能终身抗凝治疗。

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