title: [Lupus anticoagulant-hypoprothrombinemia syndrome revealing systemic lupus in an 11-year old girl in a context of clinical and biological emergency].
abstract: We report a case of lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) in an 11 year old girl initially hospitalized for bleeding. The patient presented with petechia, persisting bleeding after tooth extraction performed two days before, nephritic syndrome (renal failure, proteinuria and macroscopic hematuria), severe anemia, thrombocytopenia, lymphopenia. The association of these abnormalities suggested LAHPS secondary to severe systemic lupus. Immediate treatment with fresh frozen plasma and intravenous immunoglobulins (400 mg/kg/5d) was started and followed by steroid (500 mg/d) and cyclophosphamide (800 mg/m(2)) pulse therapy leading to rapid improvement of bleeding, renal involvement and prothrombin levels within 13 days. Lupus diagnosis was confirmed by immunological investigations and renal biopsy. Two early relapses occurred despite adequate treatment. After a follow-up of two years, no further disease activity is noted while the patient is treated only by mycophenolate mofetil (1 200 mg/m(2)/d). LAHPS did not relapse during this follow-up.
authors.affiliation: Hôpitaux de Paris, Service d'hématologie biologique, Hôpital d'enfants Armand Trousseau, Paris. remi.favier@trs.aphp.fr
authors.forename: Rémi
authors.initials: R
authors.lastname: Favier
authors.forename: Tassadit
authors.initials: T
authors.lastname: Kheyar
authors.forename: Sylvain
authors.initials: S
authors.lastname: Renolleau
authors.forename: Marie Dominique
authors.initials: MD
authors.lastname: Tabone
authors.forename: Marie
authors.initials: M
authors.lastname: Favier
authors.forename: Tim
authors.initials: T
authors.lastname: Ulinski
mesh_terms: D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications* / Q000175:diagnosis*; D002648:Child; D003937:Diagnosis, Differential; D004632:Emergency Medical Services; D005260:Female; D006470:Hemorrhage / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D006760:Hospitalization; D006801:Humans; D007020:Hypoprothrombinemias / Q000097:blood / Q000175:diagnosis* / Q000209:etiology; D016682:Lupus Coagulation Inhibitor / Q000097:blood*
chemical_list: D016682:Lupus Coagulation Inhibitor