Lupus anticoagulant-hypoprothrombinemia syndrome: A cerebral bleeding case report as systemic lupus erythematosus debut

Reumatol Clin (Engl Ed). 2023 Apr;19(4):223-227. doi: 10.1016/j.reumae.2022.02.008.

Abstract

Lupus anticoagulant-hypoprothrombinaemia syndrome (LAHPS) is a rare disorder caused by the presence of lupus anticoagulant (LA) and acquired prothrombin deficiency, which may present with severe haemorrhagic manifestations. LAHPS is usually associated with systemic lupus erythematosus (SLE), or infections and it is more frequent in the paediatric population and female gender. We describe a 42-year-old man with thrombotic antiphospholipid syndrome (APS) on chronic anticoagulation treatment with acenocoumarol who presented with spontaneous intracranial bleeding, prolongation of prothrombin time (PT), activated partial thromboplastin time (APTT) and low factor II levels (after optimal anticoagulation reversal) as a debut of SLE.

Keywords: Adult; Adulto; Hipoprotrombinemia; Hypoprothrombinaemia; Inhibidor de la coagulación lúpica; Lupus anticoagulant-hypoprothrombinaemia syndrome; Lupus coagulation inhibitor; Lupus eritematoso sistémico; Systemic lupus erythematosus; Síndrome de anticoagulante lúpico-hipoprotrombinemia.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antiphospholipid Syndrome* / complications
  • Antiphospholipid Syndrome* / diagnosis
  • Child
  • Female
  • Hemorrhage
  • Humans
  • Hypoprothrombinemias* / complications
  • Hypoprothrombinemias* / diagnosis
  • Lupus Coagulation Inhibitor
  • Lupus Erythematosus, Systemic* / complications
  • Male
  • Prothrombin

Substances

  • Lupus Coagulation Inhibitor
  • Prothrombin