Department of Pediatrics, NYU Langone Health, NYU Grossman School of Medicine, New York, NY, USA.
Department of Pediatrics, Division of Pediatric Rheumatology, Hassenfeld Children's Hospital at NYU Langone Health, NYU Grossman School of Medicine, New York, NY, USA.
Clin Rheumatol. 2023 Aug;42(8):2231-2236. doi: 10.1007/s10067-023-06619-3. Epub 2023 May 8.
Lupus anti-coagulant hypoprothrombinemia syndrome (LAHPS) is a rare condition that can be difficult to treat. It increases the risk of thrombosis and bleeding due to the presence of lupus anti-coagulant and factor II deficiency, respectively. There are a limited number of cases described in the literature. Herein we describe a case of LAHPS with bleeding symptoms as a first clinical manifestation of systemic lupus erythematosus (SLE) in an 8-year-old female. She has had multiple recurrences of her bleeding symptoms, requiring treatment with steroids, cyclophosphamide, mycophenolate mofetil, and rituximab. Her course was later complicated by development of arthritis and lupus nephritis. Her complicated course provides a new perspective on the clinical course and treatment of LAHPS. We also present a comprehensive literature review which demonstrates the difficulty in treating patients with LAHPS with underlying SLE and the variability of the clinical course and management of LAHPS depending on the age at presentation.
狼疮抗凝物致低凝血酶原血症综合征（LAHPS）是一种罕见病症，治疗较为困难。由于狼疮抗凝物和因子 II 缺乏，分别增加了血栓形成和出血的风险。文献中仅描述了有限数量的病例。在此，我们描述了一例以出血症状为首发表现的系统性红斑狼疮（SLE）的 8 岁女性 LAHPS 病例。她的出血症状多次复发，需要用类固醇、环磷酰胺、霉酚酸酯和利妥昔单抗治疗。随后，她的病情变得复杂，出现了关节炎和狼疮肾炎。她的复杂病程为 LAHPS 的临床病程和治疗提供了一个新视角。我们还进行了全面的文献回顾，表明治疗基础 SLE 合并 LAHPS 患者存在困难，且 LAHPS 的临床病程和管理因发病年龄而异。
