Lupus anticoagulant-hypoprothrombinemia syndrome presenting with co-existing cerebral venous thrombosis and subdural hemorrhage

Summary 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 (60 mg/d). Thus, anticoagulation was introduced once factor II had stabilized.