狼疮抗凝物-低凝血酶原血症综合征：以系统性红斑狼疮起病的脑溢血病例报告。

Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut.

机构信息

Department of Hematology, Hospital Universitario Fundación Jiménez Díaz, Hospitales Quirón Públicos, IIS-FJD, Madrid, Spain.

出版信息

Reumatol Clin (Engl Ed). 2023 Apr;19(4):223-227. doi: 10.1016/j.reumae.2022.02.008.

DOI:10.1016/j.reumae.2022.02.008

PMID:37061283

Abstract

Lupus anticoagulant-hypoprothrombinaemia syndrome (LAHPS) is a rare disorder caused by the presence of lupus anticoagulant (LA) and acquired prothrombin deficiency, which may present with severe haemorrhagic manifestations. LAHPS is usually associated with systemic lupus erythematosus (SLE), or infections and it is more frequent in the paediatric population and female gender. We describe a 42-year-old man with thrombotic antiphospholipid syndrome (APS) on chronic anticoagulation treatment with acenocoumarol who presented with spontaneous intracranial bleeding, prolongation of prothrombin time (PT), activated partial thromboplastin time (APTT) and low factor II levels (after optimal anticoagulation reversal) as a debut of SLE.

摘要

狼疮抗凝物-低凝血酶原血症综合征（LAHPS）是一种罕见的疾病，由狼疮抗凝物（LA）和获得性凝血酶原缺乏引起，可能表现出严重的出血症状。LAHPS 通常与系统性红斑狼疮（SLE）、感染有关，在儿科人群和女性中更为常见。我们描述了一名 42 岁男性，患有血栓性抗磷脂综合征（APS），正在接受华法林抗凝治疗，他出现自发性颅内出血，凝血酶原时间（PT）延长，活化部分凝血活酶时间（APTT）和低因子 II 水平（在最佳抗凝逆转后），这是 SLE 的首发表现。