Abstract
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: A total of 70 subjects (32 boys and 38 girls), with a mean age of 5.58 years, were included in the study. Among these subjects, 15 had autoimmune diseases (AIDs), 51 had infections, and 4 had unknown causes. Fifty-six of 70 (80%) subjects experienced bleeding with the median bleeding score of 4, 1 of 70 (1.4%) presented with thrombosis, and 13 of 70 (18.6%) were asymptomatic. All patients exhibited prolonged prothrombin time, significantly prolonged activated partial thromboplastin time, decreased factor (F)II activity (FII:C), and positive lupus anticoagulant. There was a weak negative correlation between the severity of bleeding and FII:C level (rs = -0.4283; P < .001). Patients with infection-associated LAHPS were younger than those with AIDs-associated LAHPS (P < .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.