title: 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.
authors.affiliation: aNancy University Hospital, Vascular Medicine Division and Regional Competence, Center for Rare Vascular And Systemic Autoimmune DiseasesbDijon University Hospital, Haematology laboratory, Nancy, FrancecBiochemistry, Cardiovascular Research Institute Maastricht (CARIM), Maastricht, University, Maastricht, the NetherlandsdNancy University Hospital, Nuclear Medecine & Nancyclotep Experimental Imaging, PlatformeUniversité de LorrainefInserm, UMR_S 1116gNancy University Hospital, Department of Critical Care Medicine, Nancy, France.
authors.forename: Joris
authors.initials: J
authors.lastname: Galland
authors.forename: Shirine
authors.initials: S
authors.lastname: Mohamed
authors.forename: Sabine
authors.initials: S
authors.lastname: Revuz
authors.forename: Emmanuel
authors.initials: E
authors.lastname: de Maistre
authors.forename: Bas
authors.initials: B
authors.lastname: de Laat
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Marie
authors.forename: Stéphane
authors.initials: S
authors.lastname: Zuily
authors.forename: Bruno
authors.initials: B
authors.lastname: Lévy
authors.forename: Véronique
authors.initials: V
authors.lastname: Regnault
authors.forename: Denis
authors.initials: D
authors.lastname: Wahl
mesh_terms: D000328:Adult; D017152:Antibodies, Antiphospholipid / Q000097:blood / Q000276:immunology; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006493:Heparin / Q000627:therapeutic use; D006801:Humans; D007020:Hypoprothrombinemias / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007668:Kidney / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D008099:Liver / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D016682:Lupus Coagulation Inhibitor / Q000097:blood* / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000276:immunology; D008297:Male; D009206:Myocardium / Q000276:immunology / Q000378:metabolism / Q000473:pathology; D013256:Steroids / Q000627:therapeutic use
chemical_list: D017152:Antibodies, Antiphospholipid; D016756:Immunoglobulins, Intravenous; D016682:Lupus Coagulation Inhibitor; D013256:Steroids; D006493:Heparin