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

Abstract

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.