Journal Articles Blood Coagulation & Fibrinolysis Year : 2016

Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies

Abstract

Lupus anticoagulant-hypoprothrombinemia syndrome is a rare condition characterized by the association of acquired factor II deficiency and lupus anticoagulant. Contrary to classical antiphospholipid syndrome, it may cause severe life-threatening bleeding (89% of published cases). We report a patient, positive for antidomain I antibodies, with initially primary lupus anticoagulant-hypoprothrombinemia syndrome without previous clinical manifestation or underlying systemic disease. Five years later, he experienced the first systemic lupus erythematous flare. Within a few days, catastrophic antiphospholipid syndrome was diagnosed with heart, liver and kidney involvement. The patient recovered under pulse steroids, intravenous heparin and intravenous immunoglobulins.
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Dates and versions

hal-03567151 , version 1 (11-02-2022)

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Joris Galland, Shirine Mohamed, Sabine Revuz, Emmanuel de Maistre, Bas de Laat, et al.. Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies. Blood Coagulation & Fibrinolysis, 2016, 27 (5), pp.580-582. ⟨10.1097/MBC.0000000000000545⟩. ⟨hal-03567151⟩
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