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Lupus anti-coagulant hypoprothrombinemia syndrome across different ages: a case report and review of the literature

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Abstract

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.

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Data Availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Pediatrics, NYU Langone Health, NYU Grossman School of Medicine, New York, NY, USA

    Jessica Chumsky

  2. Department of Pediatrics, Division of Pediatric Rheumatology, Hassenfeld Children’s Hospital at NYU Langone Health, NYU Grossman School of Medicine, New York, NY, USA

    Philip J. Kahn

  3. Perlmutter Cancer Center, Departments of Pediatrics and Pathology, NYU Grossman School of Medicine, New York, NY, USA

    William L. Carroll

  4. Department of Pediatrics, Hassenfeld Children’s Hospital at NYU Langone Health, NYU Grossman School of Medicine, New York, NY, USA

    Kristyn A. Pierce

  5. Department of Pediatrics, Division of Pediatric Hematology-Oncology, Hassenfeld Children’s Hospital at NYU Langone Health, NYU Grossman School of Medicine, 160 E 32Nd St., L3 Medical, New York, NY, 10016, USA

    Kirsty Hillier

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Chumsky, J., Kahn, P.J., Carroll, W.L. et al. Lupus anti-coagulant hypoprothrombinemia syndrome across different ages: a case report and review of the literature. Clin Rheumatol 42, 2231–2236 (2023). https://doi.org/10.1007/s10067-023-06619-3

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