Lupus anticoagulant-hypoprothrombinemia syndrome and catastrophic antiphospholipid syndrome in a patient with antidomain I antibodies.
Fiche publication
Date publication
juillet 2016
Journal
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis
Auteurs
Membres identifiés du Cancéropôle Est :
Pr MARIE Pierre-Yves
Tous les auteurs :
Galland J, Mohamed S, Revuz S, de Maistre E, de Laat B, Marie PY, Zuily S, Lévy B, Regnault V, Wahl D
Lien Pubmed
Résumé
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.
Mots clés
Adult, Antibodies, Antiphospholipid, blood, Antiphospholipid Syndrome, complications, Heparin, therapeutic use, Humans, Hypoprothrombinemias, complications, Immunoglobulins, Intravenous, therapeutic use, Kidney, immunology, Liver, immunology, Lupus Coagulation Inhibitor, blood, Lupus Erythematosus, Systemic, diagnosis, Male, Myocardium, immunology, Steroids, therapeutic use
Référence
Blood Coagul. Fibrinolysis. 2016 Jul;27(5):580-2