SEVERE BLEEDING DUE TO ACQUIRED HYPOPROTHROMBINEMIA LUPUS ANTICOAGULANT SYNDROME - CASE-REPORT AND REVIEW OF LITERATURE

A 17-year-old girl was admitted to our department with a hemorrhagic s yndrome due to a serious coagulopathy; prothrombin time (PT) INR was 2 .46 and the activated partial thromboplastin time (aPTT) ratio 3.46. C oagulation tests with pooled normal fresh plasma did not correct aPTT because of a coagulation inhibitor, and only partially corrected PT. F actor II activity reached only 5%. Diluted Russell viper venom test (d RVVT) and kaolin clotting time (KCT) of patient plasma (PP) and of a m ixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). The level of factor II antigen was 10%. We diagnosed systemic lupus e rythematosus (SLE) with a rare acquired hypoprothrombinemia-LA syndrom e (HLAS). The patient was treated with corticosteroids and high-dose I g and a normal PT value was re-established. (C) 1997, Ferrata Storti F oundation.