title: [Seronegative antiphospholipid syndrome, catastrophic syndrome, new anticoagulants: learning from a difficult case report].
abstract: BACKGROUND
  The diagnosis of the antiphospholipid syndrome (APS) is based on clinical and biological criteria including the persistent presence of antiphospholipid antibodies and thrombotic events or pregnancy morbidity. Heparins relayed by vitamin K antagonists (VKA) are the gold standard treatment for thrombosis.
  
  
  METHODS
  We report a 17-year-old man who presented with an initially seronegative antiphospholipid syndrome, in whom the diagnosis was late, only obtained after anticoagulation withdrawing, when a catastrophic antiphospholipid syndrome (CAPS) with cutaneous lesions and disseminated intravascular coagulation syndrome occurred. For personal convenience, this patient was initially treated with fondaparinux followed by a new oral anticoagulant (rivaroxaban) before to return to the conventional VKA treatment.
  
  
  CONCLUSIONS
  The "seronegative" APS is a controversial concept reflecting the heterogeneity of antigenic targets for aPL. This diagnosis may be considered after a rigorous work-up, with the help of haemostasis laboratories testing new emerging aPL assays. In APS, the new anticoagulants represent an attractive option needing nevertheless prospective studies to evaluate their safety and efficacy. Lupus anticoagulant detection in patients treated by new oral anticoagulants is not easy by usually recommended coagulation tests.
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
authors.forename: F
authors.initials: F
authors.lastname: Joalland
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
authors.forename: H
authors.initials: H
authors.lastname: de Boysson
authors.affiliation: Service d'hématologie biologique, hôpital européen Georges-Pompidou, AP-HP, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Darnige
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
authors.forename: A
authors.initials: A
authors.lastname: Johnson
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
authors.forename: C
authors.initials: C
authors.lastname: Jeanjean
authors.affiliation: Service d'hématologie, CHU Côte-de-Nacre, 14000 Caen, France.
authors.forename: S
authors.initials: S
authors.lastname: Cheze
authors.affiliation: Cabinet d'anatomopathologie Saint-Louis, 14, rue Gaston-Lavalley, 14000 Caen, France.
authors.forename: A
authors.initials: A
authors.lastname: Augustin
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France.
authors.forename: C
authors.initials: C
authors.lastname: Auzary
authors.affiliation: Service de médecine interne, centre hospitalier Robert-Bisson, 4, rue Roger-Aini, 14100 Lisieux, France. Electronic address: l.geffray@ch-lisieux.fr.
authors.forename: L
authors.initials: L
authors.lastname: Geffray
mesh_terms: D000293:Adolescent; D017152:Antibodies, Antiphospholipid / Q000097:blood*; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy / Q000209:etiology*; D065427:Factor Xa Inhibitors / Q000627:therapeutic use; D000077425:Fondaparinux; D006801:Humans; D008297:Male; D009025:Morpholines / Q000627:therapeutic use; D011134:Polysaccharides / Q000627:therapeutic use; D000069552:Rivaroxaban; D013876:Thiophenes / Q000627:therapeutic use
chemical_list: D017152:Antibodies, Antiphospholipid; D000925:Anticoagulants; D065427:Factor Xa Inhibitors; D009025:Morpholines; D011134:Polysaccharides; D013876:Thiophenes; D000069552:Rivaroxaban; D000077425:Fondaparinux
keywords: Antiphospholipid syndrome; Catastrophic antiphospholipid syndrome; Coagulation intravasculaire disséminée; Disseminated intravascular coagulation syndrome; New oral anticoagulants; Nouveaux anticoagulants oraux; Syndrome catastrophique des antiphospholipides; Syndrome des antiphospholipides