[Acquired haemophilia: Update in 2024].

Autor: Lévesque H; Service de médecine interne, CHU de Rouen, Normandie université, UNIROUEN, U 1096, 1, rue de Germont, 76000 Rouen, France. Electronic address: herve.levesque@chu-rouen.fr., Guillet B; Centre de référence de l'hémophilie et des maladies hémorragiques, constitutionnelles, CHU de Rennes, Rennes, France; EHESP, Institut de recherche en santé, environnement et travail (IRSET) - UMR-S 1085, CHU de Rennes, université de Rennes, Inserm, 35000 Rennes, France., d'Oiron R; Centre de référence de l'hémophilie et des maladies hémorragiques, constitutionnelles, hôpital Bicêtre, AP-HP, HITh, UMR-S1176, Inserm, université Paris Saclay, Le Kremlin-Bicêtre, Paris, France., Benhamou Y; Service de médecine interne, CHU de Rouen, Normandie université, UNIROUEN, U 1096, 1, rue de Germont, 76000 Rouen, France.
Jazyk: francouzština
Zdroj: La Revue de medecine interne [Rev Med Interne] 2024 Nov; Vol. 45 (11), pp. 710-725. Date of Electronic Publication: 2024 Sep 07.
DOI: 10.1016/j.revmed.2024.06.005
Abstrakt: Acquired hemophilia A (AHA) is a rare autoimmune disorder due to autoantibodies against factor VIII, with a high mortality risk. It should be suspected in subjects with abnormal bleedings, especially subcutaneous bleed associated with prolonged activated partial thromboplastin time (aPTT). AHA is often idiopathic but is associated with autoimmune diseases, malignancies, pregnancy and postpartum period or drugs. Treatment is based on haemostatic agents as by-passants agents such as factor VIIa and activated prothrombine concentrate complex or recombinant porcine factor VIII for severe bleeding. Eradication of inhibitor should be established as soon as the diagnosis is confirmed with steroid alone often associated with cytotoxic agents or rituximab, depending on FVIII activity and inhibitor titer. The purpose of this review is to summarize the epidemiology, etiopathogenesis, diagnosis, treatment of AHA and discuss current recommendations.
(Copyright © 2024. Published by Elsevier Masson SAS.)
Databáze: MEDLINE
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