Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature.

Abstract

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.

• Authors: S M N Mulliez, F De Keyser, C Verbist, A Vantilborgh, W Wijns, I Beukinga, K M J Devreese
• Journal: Lupus (Lupus) Vol. 24 Issue 7 Pg. 736-45 (Jun 2015) ISSN: 1477-0962 [Electronic] England
• PMID: 25391540 (Publication Type: Case Reports, Journal Article, Review)
• Copyright: © The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav.

Chemical References

• Antibodies, Antiphospholipid
• Lupus Coagulation Inhibitor

Topics

• Adult
• Antibodies, Antiphospholipid (blood)
• Antiphospholipid Syndrome (blood)
• Child, Preschool
• Female
• Humans
• Hypoprothrombinemias (blood)
• Lupus Coagulation Inhibitor (blood)
• Lupus Erythematosus, Systemic (blood)
• Male