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 syndrome due to a serious coagulopathy; prothrombin time (PT) INR was 2.46 and the activated partial thromboplastin time (aPTT) ratio 3.46. Coagulation tests with pooled normal fresh plasma did not correct aPTT because of a coagulation inhibitor, and only partially corrected PT. Factor II activity reached only 5%. Diluted Russell viper venom tests (dRVVT) and kaolin clotting time (KCT) of patient plasma (PP) and of a mixture of PP/normal plasma (NP) detected the lupus anticoagulant (LA). The level of factor II antigen was 10%. We diagnosed systemic lupus erythematosus (SLE) with a rare acquired hypoprothrombinemia-LA syndrome (HLAS). The patient was treated with corticosteroids and high-dose Ig and a normal PT value was re-established.
Vivaldi
P
P
Department of Medicine II, S. Chiara General Hospital, Trento, Italy.
Rossetti
G
G
Galli
M
M
Finazzi
G
G
D000293:Adolescent; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000628:therapy; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000628:therapy; D001780:Blood Coagulation Tests; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D007020:Hypoprothrombinemias / Q000209:etiology* / Q000628:therapy; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016682:Lupus Coagulation Inhibitor / Q000032:analysis* / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications* / Q000276:immunology / Q000628:therapy; D011239:Prednisolone / Q000627:therapeutic use; D011516:Prothrombin / Q000276:immunology
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D016682:Lupus Coagulation Inhibitor; D011516:Prothrombin; D011239:Prednisolone