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

Clin Rheumatol. 2023 Aug;42(8):2231-2236. doi: 10.1007/s10067-023-06619-3. Epub 2023 May 8.

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.

Keywords: Anti-phospholipid syndrome; Bleeding; Systemic lupus erythematosus; Thromboses.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Female
  • Hemorrhage / complications
  • Humans
  • Hypoprothrombinemias* / complications
  • Hypoprothrombinemias* / diagnosis
  • Hypoprothrombinemias* / drug therapy
  • Lupus Coagulation Inhibitor
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / diagnosis
  • Lupus Erythematosus, Systemic* / drug therapy

Substances

  • Lupus Coagulation Inhibitor
  • Cyclophosphamide