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

par Mulliez, Sylvie M N;De Keyser, Filip;Verbist, Christophe;Vantilborgh, Anna;Wijns, Walter ;Beukinga, Ingrid ;Devreese, Katrien
Référence Lupus, 24, 7, page (736-745)
Publication Publié, 2015-06

Article révisé par les pairs

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Résumé :

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.