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抗磷脂抗体综合征。

Antiphospholipid antibody syndrome.

机构信息

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

出版信息

Hematology Am Soc Hematol Educ Program. 2009:233-9. doi: 10.1182/asheducation-2009.1.233.

Abstract

The antiphospholipid antibody syndrome (APS) is defined by the persistent presence of antiphospholipid antibodies in patients with recurrent venous or arterial thromboembolism or pregnancy morbidity. Anti-thrombotic therapy is the mainstay of treatment given the high risk of recurrent thromboembolism that characterizes this condition. Despite the prothrombotic nature of APS, thrombocytopenia is present in a proportion of patients. which can complicate management and limit the use of antithrombotic therapy. The mechanism of APS-associated thrombocytopenia is multifactorial and its relation to thrombotic risk poorly characterized. However, the presence of thrombocytopenia does not appear to reduce thrombotic risk in patients with APS, who can develop thromboembolic complications necessitating antithrombotic treatment. In these cases, treatment of the thrombocytopenia may be necessary to facilitate administration of antithrombotic agents. Clinical trials have demonstrated that patients with antiphospholipid antibodies and venous thromboembolism should be treated with vitamin K antagonists (warfarin); that ischemic stroke may be treated with aspirin or warfarin; and that women with recurrent pregnancy loss should receive prophylactic-dose heparin and aspirin. However, application of these trial results to patients with APS-associated thrombocytopenia can be challenging since there are limited data on the optimal use of antithrombotic agents in this setting. Issues such as determining the platelet threshold at which antithrombotic agents can be safely used and managing patients with both bleeding and thromboembolic complications remain unresolved. Ultimately the risks and benefits of antithrombotic therapy, balanced against the severity of the thrombocytopenia and its potential bleeding risks, need to be assessed using an individualized patient approach.

摘要

抗磷脂抗体综合征(APS)的定义为患者存在持续的抗磷脂抗体,伴有复发性静脉或动脉血栓栓塞或妊娠并发症。鉴于这种情况具有高复发血栓栓塞的风险,抗血栓治疗是主要的治疗方法。尽管 APS 具有促血栓形成的性质,但一部分患者存在血小板减少症。这可能会使管理复杂化并限制抗血栓治疗的使用。APS 相关血小板减少症的发病机制是多因素的,其与血栓形成风险的关系尚未明确。然而,血小板减少症的存在似乎不会降低 APS 患者的血栓形成风险,这些患者可能会出现需要抗血栓治疗的血栓栓塞并发症。在这些情况下,可能需要治疗血小板减少症以方便给予抗血栓药物。临床试验表明,患有抗磷脂抗体和静脉血栓栓塞的患者应使用维生素 K 拮抗剂(华法林)治疗;缺血性中风可能用阿司匹林或华法林治疗;复发性妊娠丢失的女性应接受预防性剂量的肝素和阿司匹林治疗。然而,将这些试验结果应用于 APS 相关血小板减少症患者可能具有挑战性,因为在这种情况下,关于抗血栓药物的最佳使用的数据有限。诸如确定可以安全使用抗血栓药物的血小板阈值以及管理同时存在出血和血栓栓塞并发症的患者等问题仍未解决。最终,需要使用个体化患者方法来评估抗血栓治疗的风险和益处,平衡血小板减少症的严重程度及其潜在的出血风险。

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