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一名9岁女孩的狼疮抗凝物与短暂性严重出血倾向

Lupus anticoagulant and transient severe bleeding tendency in a girl aged nine years.

作者信息

Nanulescu M, Trif I, Cucuianu M, Rus H G, Cristea A, Niculescu F

机构信息

1 Clinic of Pediatrics, Cluj-Napoca, Romania.

出版信息

Med Interne. 1988 Apr-Jun;26(2):115-9.

PMID:3133747
Abstract

A 9-year old girl admitted in our clinic for severe hemorrhagic syndrome was found to display a high level of lupus anticoagulant (LA) leading to an important prolongation of phospholipid-dependent coagulation. Positive antinuclear and anti DNA antibodies, as well as very low levels of complement C3 and C4 proteins confirmed the diagnosis of systemic lupus erythematosus. Therapy with cortisone and cyclophosphamide led to normalization of the clotting tests but could not arrest the development of renal and hepatic lesions. The patient is one of the few cases with presence of lupus anticoagulant associated with severe hemorrhagic diathesis, in opposition to the more frequently reported thrombotic tendency connected with antiphospholipid antibodies.

摘要

一名9岁女童因严重出血综合征入住我院,检查发现其狼疮抗凝物(LA)水平很高,导致磷脂依赖性凝血显著延长。抗核抗体和抗DNA抗体呈阳性,以及补体C3和C4蛋白水平极低,确诊为系统性红斑狼疮。使用可的松和环磷酰胺治疗后凝血试验恢复正常,但未能阻止肾和肝损伤的发展。该患者是少数伴有狼疮抗凝物且有严重出血素质的病例之一,与更常见的抗磷脂抗体相关血栓形成倾向相反。

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