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

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

https://www.ncbi.nlm.nih.gov/pubmed/27380476

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