title: Lupus anticoagulant-hypoprothrombinemia syndrome presenting with co-existing cerebral venous thrombosis and subdural hemorrhage.
abstract: The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count. Evaluation of the clotting factors revealed decreased levels of factors II (37%). Factors V, VIII, IX and XI were normal. Lupus anticoagulant (LA) was demonstrated by the Dilute Russell's Viper Venom Test (DRVVT). Immunological work-up was positive for IgG type anticardiolipines antibodies (aCL). Successful management consisted first of oral prednisone (60mg/d). Thus, anticoagulation was introduced once factor II had stabilized.
authors.affiliation: Service de médecine interne, CHU La Rabta, Tunis, Tunisia. Electronic address: belfeki.nabil@gmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Bel Feki
authors.affiliation: Service des maladies infectieuses, CHU La Rabta, Tunis, Tunisia.
authors.forename: S
authors.initials: S
authors.lastname: Zayet
authors.affiliation: Service de médecine interne, CHU La Rabta, Tunis, Tunisia.
authors.forename: I
authors.initials: I
authors.lastname: Ben Ghorbel
authors.affiliation: Service de médecine interne, CHU La Rabta, Tunis, Tunisia.
authors.forename: M-H
authors.initials: MH
authors.lastname: Houman
mesh_terms: D000328:Adult; D016736:Antiphospholipid Syndrome / Q000150:complications; D002550:Cerebral Veins; D005260:Female; D006408:Hematoma, Subdural / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007020:Hypoprothrombinemias / Q000097:blood / Q000175:diagnosis* / Q000209:etiology; D020767:Intracranial Thrombosis / Q000150:complications / Q000175:diagnosis*; D016682:Lupus Coagulation Inhibitor / Q000009:adverse effects / Q000032:analysis*; D010314:Partial Thromboplastin Time; D011241:Prednisone / Q000627:therapeutic use; D011516:Prothrombin / Q000032:analysis; D011517:Prothrombin Time
chemical_list: D016682:Lupus Coagulation Inhibitor; D011516:Prothrombin; D011241:Prednisone
keywords: Antiphospholipid syndrome; Hypoprothrombinemia; Hypoprothrombinémie; Lupus anticoagulant; Syndrome des antiphospholipides