title: Lupus anticoagulant-hypoprothrombinemia syndrome: report of 8 cases and review of the literature.
abstract: 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.
authors.affiliation: 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.
authors.forename: Karin
authors.initials: K
authors.lastname: Mazodier
authors.forename: Laurent
authors.initials: L
authors.lastname: Arnaud
authors.forename: Alexis
authors.initials: A
authors.lastname: Mathian
authors.forename: Nathalie
authors.initials: N
authors.lastname: Costedoat-Chalumeau
authors.forename: Julien
authors.initials: J
authors.lastname: Haroche
authors.forename: Camille
authors.initials: C
authors.lastname: Frances
authors.forename: Jean-Robert
authors.initials: JR
authors.lastname: Harlé
authors.forename: Gilles
authors.initials: G
authors.lastname: Pernod
authors.forename: Eric
authors.initials: E
authors.lastname: Lespessailles
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaudin
authors.forename: Hilaire
authors.initials: H
authors.lastname: Charlanne
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones; D000328:Adult; D000368:Aged; D016736:Antiphospholipid Syndrome / Q000150:complications* / Q000175:diagnosis; D001327:Autoimmune Diseases / Q000150:complications; D005260:Female; D005602:France; D006801:Humans; D007020:Hypoprothrombinemias / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D016682:Lupus Coagulation Inhibitor; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D011516:Prothrombin / Q000276:immunology*; D012189:Retrospective Studies
chemical_list: D000305:Adrenal Cortex Hormones; D016682:Lupus Coagulation Inhibitor; D011516:Prothrombin