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- Title
Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature.
- Authors
Mulliez, S M N; De Keyser, F; Verbist, C; Vantilborgh, A; Wijns, W; Beukinga, I; Devreese, K M J
- 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.
- Subjects
ANTICOAGULANTS; RARE diseases; PROTHROMBIN; SYSTEMIC lupus erythematosus; DISEASES in women; HEMORRHAGE
- Publication
Lupus, 2015, Vol 24, Issue 7, p736
- ISSN
0961-2033
- Publication type
Article
- DOI
10.1177/0961203314558859