Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature
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- S M N Mulliez
- Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, Belgium
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- F De Keyser
- Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
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- C Verbist
- Department of Rheumatology, Ghent University Hospital, Ghent, Belgium
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- A Vantilborgh
- Department of Hematology, Ghent University Hospital, Ghent, Belgium
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- W Wijns
- Department of Hematobiology, Erasme Hospital, Brussels, Belgium
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- I Beukinga
- Department of Hematobiology, Erasme Hospital, Brussels, Belgium
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- K M J Devreese
- Coagulation Laboratory, Department of Clinical Chemistry, Microbiology and Immunology, Ghent University Hospital, Ghent, Belgium
説明
<jats:p> 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. </jats:p>
収録刊行物
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- Lupus
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Lupus 24 (7), 736-745, 2014-11-12
SAGE Publications