Article Dans Une Revue Thrombosis Research Année : 2025

Lupus-associated hypoprothrombinemia syndrome in children: Differences between post-infectious and autoimmune forms

Sébastien Héritier

Résumé

Introduction: Lupus-anticoagulant hypoprothrombinemia syndrome (LAHS) is a rare but potentially serious condition. LAHS can be of post-infectious (PI) or autoimmune (AI) origin. However, there is currently no clear data available on the differences between these two forms. Method: A retrospective multicenter study of cases in France was performed, followed by a review of cases in the literature. Result: A total of 84 patients were included in the study. Seventeen patients were selected from the French cohort, and 67 were selected from a systematic review of the literature. 95 % of patients presented with hemorrhagic symptoms, with nearly half of these cases being severe. PI or AI context was identified in 33 % and 53 % of cases. 54 % of patients were treated with corticosteroids, and 30 % received immunomodulatory therapy. Thrombopenia and lower factor V were associated with a higher risk of bleeding. The AI group consisted of older children and exhibited significantly more severe bleeding (p < 0.001). The treatment was more frequent and intensive, and the relapse rate was higher in the AI group (p < 0.001). Conclusion: Post-infectious forms are transient and associated with a low risk of serious hemorrhage. The treatment must be adapted according to the clinical and biological context.

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Dates et versions

hal-04951795 , version 1 (17-02-2025)

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Hanna Zighed, Yoann Huguenin, Laurence Blanc, Jean-Baptiste Valentin, Antoine Babuty, et al.. Lupus-associated hypoprothrombinemia syndrome in children: Differences between post-infectious and autoimmune forms. Thrombosis Research, 2025, 245, pp.109231. ⟨10.1016/j.thromres.2024.109231⟩. ⟨hal-04951795⟩
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