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<p>Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare, acquired coagulation disorder where antiphospholipid antibodies (aPL) target prothrombin. LAHPS should be suspected in patients with aPL presenting with bleeding rather than thrombotic events or prolonged prothrombin time. Prompt assessment of factor II levels is crucial to prevent life-threatening hemorrhage. Given its rarity, there is currently no established treatment guidelines. Management of bleeding episodes involves transfusion of fresh frozen plasma or prothrombin complex concentrate (PPC), activated factor concentrates such as FEIBA or recombinant factor VIIa could be considered if initial interventions fail. Concurrent immunosuppressive therapy is essential to suppress pathogenic anti-prothrombine antibodies, with corticosteroids typically serving as first-line treatment, either alone or in combination with other immunosuppressive agents. Here, we present a case of a newly diagnosed LAHPS in a patient with active systemic lupus erythematosus who was successively treated with PCCs and immunosuppression with corticosteroids and rituximab.</p>

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Lupus anticoagulant hypoprothrombinemia syndrome successfully treated with rituximab – case report

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年2月24日

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  • Michael Iarossi,
  • Angelo Rizzolo,
  • Michèle Stanciu,
  • Valérie Leclair,
  • Marc Blostein,
  • Maral Koolianl

摘要

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare, acquired coagulation disorder where antiphospholipid antibodies (aPL) target prothrombin. LAHPS should be suspected in patients with aPL presenting with bleeding rather than thrombotic events or prolonged prothrombin time. Prompt assessment of factor II levels is crucial to prevent life-threatening hemorrhage. Given its rarity, there is currently no established treatment guidelines. Management of bleeding episodes involves transfusion of fresh frozen plasma or prothrombin complex concentrate (PPC), activated factor concentrates such as FEIBA or recombinant factor VIIa could be considered if initial interventions fail. Concurrent immunosuppressive therapy is essential to suppress pathogenic anti-prothrombine antibodies, with corticosteroids typically serving as first-line treatment, either alone or in combination with other immunosuppressive agents. Here, we present a case of a newly diagnosed LAHPS in a patient with active systemic lupus erythematosus who was successively treated with PCCs and immunosuppression with corticosteroids and rituximab.