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抗磷脂抗体综合征问题

The Issue of the Antiphospholipid Antibody Syndrome.

作者信息

Oliveira Dinaldo C, Correia Augusto, Oliveira Carolina

机构信息

Hospital das Clinicas, Federal University of Pernambuco, Recife-PE, Brazil.

出版信息

J Clin Med Res. 2020 May;12(5):286-292. doi: 10.14740/jocmr4154. Epub 2020 May 8.

DOI:10.14740/jocmr4154
PMID:32489503
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7239578/
Abstract

Antiphospholipid antibody syndrome (APS) is a state of hypercoagulability secondary to an autoimmune disorder. It is associated with thrombotic events in venous and arterial vessels, obstetric complications characterized by recurrent fetal losses, and increased perinatal morbidity. APS is classified as primary, when not associated with other pathologies; or secondary, when associated with an underlying autoimmune disease with, solid tumor, or hematological disorder. Clinical findings include livedo reticularis, thrombocytopenia or hemolytic anemia, maternal morbidity, and recurrent thrombotic episodes and others. Laboratory tests show circulating antiphospholipid antibodies (aPLs); however, even in the presence of these antibodies, patients can be asymptomatic. Estimates predict that about 5% of the populations have circulating aPLs, but the incidence of APS is only five cases per 100,000 people, as diagnosis of this syndrome requires clinical and laboratory findings to be simultaneously present. In cases of secondary APS, or in acute cases with imminent risk of death (as in catastrophic APS), it may be necessary to reduce aPL serum levels using immunomodulators, immunosuppressants, or plasmapheresis, in order to treat the associated pathologies. In other situations, the use of immunotherapy is not indicated. In other patients heparin, aspirin or anticoagulants either alone or associated should be administered depending on each specific case.

摘要

抗磷脂抗体综合征(APS)是一种继发于自身免疫性疾病的高凝状态。它与静脉和动脉血管的血栓形成事件、以反复流产为特征的产科并发症以及围产期发病率增加有关。APS分为原发性(不伴有其他病理情况)或继发性(与潜在的自身免疫性疾病、实体瘤或血液系统疾病相关)。临床表现包括网状青斑、血小板减少或溶血性贫血、孕产妇发病以及反复血栓形成发作等。实验室检查显示循环抗磷脂抗体(aPLs);然而,即使存在这些抗体,患者也可能无症状。据估计,约5%的人群有循环aPLs,但APS的发病率仅为每10万人中有5例,因为该综合征的诊断需要临床和实验室检查结果同时出现。在继发性APS病例中,或在有迫在眉睫的死亡风险的急性病例中(如灾难性APS),可能有必要使用免疫调节剂、免疫抑制剂或血浆置换来降低aPL血清水平,以治疗相关疾病。在其他情况下,不建议使用免疫疗法。在其他患者中,应根据具体情况单独或联合使用肝素、阿司匹林或抗凝剂。

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Antiphospholipid syndrome: an update for clinicians and scientists.抗磷脂综合征:临床医生和科学家的最新进展
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Antiphospholipid Syndrome: Role of Vascular Endothelial Cells and Implications for Risk Stratification and Targeted Therapeutics.抗磷脂综合征:血管内皮细胞的作用及其对风险分层和靶向治疗的意义。
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Pathogenesis and management of antiphospholipid syndrome.抗磷脂综合征的发病机制与管理
一例以冠状动脉支架内再狭窄为主要表现的抗磷脂抗体综合征。
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The trends in the incidence and thrombosis-related comorbidities of antiphospholipid syndrome: a 14-year nationwide population-based study.抗磷脂综合征的发病率及血栓形成相关合并症的趋势:一项基于全国人群的14年研究
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