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: Karin Mazodier, Laurent Arnaud, Alexis Mathian, Nathalie Costedoat-Chalumeau, Julien Haroche, Camille Frances, Jean-Robert Harlé, Gilles Pernod, Eric Lespessailles, Philippe Gaudin, Hilaire Charlanne, Eric Hachulla, Patrick Niaudet, Jean-Charles Piette, Zahir Amoura
• Journal: Medicine (Medicine (Baltimore)) Vol. 91 Issue 5 Pg. 251-260 (Sep 2012) ISSN: 1536-5964 [Electronic] United States
• PMID: 22932789 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Adrenal Cortex Hormones
• Lupus Coagulation Inhibitor
• Prothrombin

Topics

• Adolescent
• Adrenal Cortex Hormones
• Adult
• Aged
• Antiphospholipid Syndrome (complications, diagnosis)
• Autoimmune Diseases (complications)
• Female
• France
• Humans
• Hypoprothrombinemias (complications, diagnosis, immunology)
• Lupus Coagulation Inhibitor
• Lupus Erythematosus, Systemic (complications, diagnosis)
• Male
• Middle Aged
• Prothrombin (immunology)
• Retrospective Studies