[Acquired hemophilia A: clinical and biological characteristics and therapeutic management of a series of eight patients hospitalized in Lariboisière and Saint-Louis hospitals].
Hémophilie A acquise : caractéristiques clinico-biologiques et prise en charge thérapeutique d’une série de huit patients hospitalisés à Lariboisière et Saint-Louis.
FVIII autoantibodies
acquired haemophilia A
bleeding
haemostatic agent
immunosuppressive therapy
Journal
Annales de biologie clinique
ISSN: 1950-6112
Titre abrégé: Ann Biol Clin (Paris)
Pays: France
ID NLM: 2984690R
Informations de publication
Date de publication:
01 Feb 2021
01 Feb 2021
Historique:
pubmed:
25
2
2021
medline:
26
10
2021
entrez:
24
2
2021
Statut:
ppublish
Résumé
Acquired hemophilia A is a rare autoimmune disease, linked to the appearance of autoantibodies directed against circulating factor VIII, and characterized by a major hemorrhagic syndrome. Acquired hemophilia A is a life-threatening diagnostic and therapeutic medical emergency. We describe here the cohort of patients with acquired hemophilia A treated between 2015 and 2020 at Lariboisière and Saint-Louis University Hospitals (Paris, France). We remind you here of the measures to be taken without delay in the face of any clinical and/or biological suspicion. Management is based on three main areas published in multicentre cohort studies, essentially observational: symptomatic treatment to control the hemorrhagic syndrome, immunosuppressive treatment to eradicate autoantibodies and manage their possible complications, and etiological treatment of the underlying pathology for secondary forms.
Identifiants
pubmed: 33622662
pii: abc.2021.1625
doi: 10.1684/abc.2021.1625
doi:
Substances chimiques
Autoantibodies
0
Immunosuppressive Agents
0
Factor VIII
9001-27-8
Types de publication
Journal Article
Langues
fre
Sous-ensembles de citation
IM