Lupus-associated hypoprothrombinemia syndrome in children: Differences between post-infectious and autoimmune forms
Zighed Hanna a), b), Huguenin Yoann c), Blanc Laurence d), Valentin Jean-Baptiste e), Babuty Antoine f), Cussac Vincent g), Heritier Sebastien h), Biron-Andreani Christine i), Jeziorski Eric b), Moulis Lionel j), Harroche Annie k), Theron Alexandre a), i), l)
a) Department of Pediatric Oncology and Hematology, Univ Montpellier, CHU Montpellier, Montpellier, France
b) Department of General Pediatrics, Infectiology, and Clinical Immunology, CHU Montpellier, Montpellier, France
c) Resources and Competence Centre for Constitutional Bleeding Disorders, Bordeaux University Hospital, Bordeaux, France
d) Paediatric Haematology-Oncology Unit, Poitiers University Hospital, Poitiers, France
e) Ressources and Competence Centre for Constitutional Bleeding Disorders, Tours University Hospital, Tours, France
f) Hematology Laboratory, Nantes University Hospital, Nantes, France
g) Hematology Laboratory, Le Mans Hospital, Le Mans, France
h) Pediatric Hematology Oncology Unit, Armand Trousseau Hospital, AP-HP, Sorbonne Université, Paris, France
i) Resources and Competence Center for Constitutional Bleeding Disorders, Univ Montpellier, CHU Montpellier, Montpellier, France
j) Clinical Research and Epidemiology Unit, Department of Public Health, Univ Montpellier, CHU Montpellier, Montpellier, France
k) Ressources and Competence Centre for Constitutional Bleeding Disorders, Necker Hospital, APHP, Paris, France
l) IRMB, University of Montpellier, INSERM, Montpellier, France
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.