Nusrat Sanober, Tewari Sayani, Khan Osman
Hematology-Oncology Section, Department of Medicine, University of Oklahoma Health Sciences Center, 800 NE 10th Street, Oklahoma City, OK, 73104, USA.
Nemours Children's Health, Seaford, Delaware, USA.
Thromb J. 2023 Jul 17;21(1):77. doi: 10.1186/s12959-023-00517-z.
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare acquired bleeding disorder secondary to development of antibodies against prothrombin protein, in the presence of antiphospholipid antibodies. We describe the case of a 13-year-old girl who presented with severe menorrhagia and symptomatic anemia. Labs indicated anemia, thrombocytopenia, elevated PT and aPTT, high-titer inhibitor on mixing studies, positive ANA and anti-dsDNA antibodies, along with a triple-positive antiphospholipid antibody panel. Given additional systemic manifestations, systemic lupus erythematosus was diagnosed. High dose steroids and hydroxychloroquine subsequently started. Her clinical course was complicated by femoral deep venous thrombosis and post renal biopsy retroperitoneal hematoma. Further workup revealed low prothrombin level and the diagnosis of lupus anticoagulant hypoprothrombinemia syndrome. In view of suboptimal response to initial immunosuppressive therapy, rituximab was added to her regimen, leading to an improvement in clinical symptoms and resolution of hypoprothrombinemia. She remains recurrence free 5 years from the event.
狼疮抗凝物-低凝血酶原血症综合征（LAHPS）是一种罕见的获得性出血性疾病，继发于在抗磷脂抗体存在的情况下针对凝血酶原蛋白产生抗体。我们描述了一名13岁女孩的病例，她出现严重月经过多和症状性贫血。实验室检查显示贫血、血小板减少、凝血酶原时间（PT）和活化部分凝血活酶时间（aPTT）升高，混合试验中有高滴度抑制剂，抗核抗体（ANA）和抗双链DNA抗体阳性，以及抗磷脂抗体三项阳性。鉴于还有其他全身表现，诊断为系统性红斑狼疮。随后开始使用高剂量类固醇和羟氯喹。她的临床病程因股深静脉血栓形成和肾活检后腹膜后血肿而复杂化。进一步检查发现凝血酶原水平低，诊断为狼疮抗凝物低凝血酶原血症综合征。鉴于对初始免疫抑制治疗反应欠佳，在她的治疗方案中加用了利妥昔单抗，导致临床症状改善和低凝血酶原血症消退。自发病以来5年，她未再复发。
