Clinical features and treatment of 70 children with lupus anticoagulant-hypoprothrombinemia syndrome: a retrospective study from a single center in China

Background: Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare acquired bleeding disorder characterized by the presence of lupus anticoagulant (LA) and acquired hypoprothrombinemia. Objectives: To summarize the experience of diagnosis, clinical features, and treatment of lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS). Methods: A retrospective study of 70 children diagnosed with LAHPS from January 2019 to February 2024 at a single center was conducted. Results: < .0001). In the study, LAHPS subjects are treated with corticosteroids as the first-line therapy, or in combination with immunosuppressants. Coagulation factor replacement therapy can effectively prevent and control bleeding events. After follow-up, lupus anticoagulant of all patients had turned negative within 12 weeks. And, prothrombin time and FII:C were completely normalized of all patients without recurrence of bleeding and without thrombosis. Conclusion: Children develop LAHPS most commonly after AIDs and infection. Most patients presented with mild to moderate bleeding. The severity of bleeding symptoms was not exactly parallel to the decreased FII:C level.