[Hypoprothrombinemia-lupus anticoagulant syndrome: a case report and literature review].

Abstract

The Hypoprothrombinemia-Lupus Anticoagulant Syndrome (HLAS) is a rare coagulation disorder, typically presenting with bleeding manifestations. It is characterized by decreased prothrombin activity and the presence of lupus anticoagulant, but laboratory findings are complex, which can delay diagnosis and treatment. This report describes the diagnosis and management of a HLAS patient who was an 11-year-old girl with recurrent bleeding. Coagulation tests revealed prolonged APTT and PT, decreased prothrombin activity, and immediate inhibitory antibodies in the APTT correction test. Lupus anticoagulant, antiphospholipid antibodies, anti-β(2)-glycoprotein 1 antibodies, and phosphatidylserine/prothrombin (aPS/PT) antibodies were positive, leading to a diagnosis of Hypoprothrombinemia-Lupus Anticoagulant Syndrome. The patient was also diagnosed with systemic lupus erythematosus. Following corticosteroid therapy, prothrombin activity returned to normal.