Mazodier Karin, Arnaud Laurent, Mathian Alexis, Costedoat-Chalumeau Nathalie, Haroche Julien, Frances Camille, Harlé Jean-Robert, Pernod Gilles, Lespessailles Eric, Gaudin Philippe, Charlanne Hilaire, Hachulla Eric, Niaudet Patrick, Piette Jean-Charles, Amoura Zahir
From the Service de Médecine Interne (KM, JRH), Hôpital La Conception, Marseille; Service de Médecine Interne (LA, AM, NCC, JH, CF, JCP, ZA), Hôpital Pitié-Salpêtrière and Université Pierre et Marie Curie, Paris; Laboratoire d'Hématologie (GP), CHU de Grenoble, Grenoble; Service de Rhumatologie (EL), CHR d'Orléans, Orléans; Service de Rhumatologie (PG), CHUG Hôpital Sud, Grenoble; Service de Médecine Interne (HC, EH), Hôpital Claude Huriez, Lille; and Service de Néphro-Pédiatrie (PN), Hôpital Necker, Paris; France.
Medicine (Baltimore). 2012 Sep;91(5):251-260. doi: 10.1097/MD.0b013e31826b971f.
The lupus anticoagulant-hypoprothrombinemia syndrome (LAHS)--the association of acquired factor II deficiency and lupus anticoagulant--is a rare disease drastically different from antiphospholipid syndrome in that it may cause predisposition not only to thrombosis but also to severe bleeding. We performed a retrospective study of 8 patients with LAHS referred to 6 French tertiary care centers between January 2003 and February 2011, and a literature review retrieving all related articles published between 1960 and April 2011. Including our 8 new cases, LAHS has been reported in 74 cases. The disease mostly occurs in young adults, with a female to male sex ratio of 1.4. Associated conditions mostly include autoimmune diseases such as systemic lupus erythematosus and infectious diseases. Bleeding is a frequent feature (89% of cases), while arterial and/or venous thrombosis is less common (13%). Factor II level is severely decreased at diagnosis (median value, 11%; range, 1%-40%). LAHS associated with autoimmune diseases is more persistent than LAHS associated with infection, and hemorrhagic complications are more common. Corticosteroids should be considered the first-line treatment, but the thrombotic risk strongly increases during treatment because of the improvement of factor II level. Despite the fact that 50% of patients develop severe bleeding, the mortality rate is <5%, after a median follow-up of 13 months (range, 0.5-252 mo). LAHS associated with autoimmune diseases should be diagnosed and managed carefully because the disease is persistent and severe hemorrhagic complications are common.
狼疮抗凝物-低凝血酶原血症综合征（LAHS）——获得性凝血因子II缺乏与狼疮抗凝物的关联——是一种罕见疾病，与抗磷脂综合征截然不同，因为它不仅可能导致血栓形成倾向，还可能导致严重出血。我们对2003年1月至2011年2月期间转诊至6家法国三级医疗中心的8例LAHS患者进行了回顾性研究，并对1960年至2011年4月期间发表的所有相关文章进行了文献综述。包括我们的8例新病例在内，LAHS已报告74例。该疾病多发生于年轻人，男女比例为1.4。相关疾病主要包括自身免疫性疾病如系统性红斑狼疮和感染性疾病。出血是常见特征（89%的病例），而动脉和/或静脉血栓形成较少见（13%）。诊断时凝血因子II水平严重降低（中位数为11%；范围为1%-40%）。与自身免疫性疾病相关的LAHS比与感染相关的LAHS更持久，出血并发症更常见。应将糖皮质激素视为一线治疗，但由于凝血因子II水平的改善，治疗期间血栓形成风险会大幅增加。尽管50%的患者会发生严重出血，但中位随访13个月（范围为0.5-252个月）后死亡率<5%。与自身免疫性疾病相关的LAHS应仔细诊断和管理，因为该疾病持续存在且严重出血并发症常见。
