Les lymphohistiocytoses hémophagocytaires [Hemophagocytic Lymphohistiocytosis]

Détails

ID Serval
serval:BIB_D46E28896440
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Les lymphohistiocytoses hémophagocytaires [Hemophagocytic Lymphohistiocytosis]
Périodique
Praxis
Auteur(s)
Stalder G., Ribi C., Duchosal M.A.
ISSN
1661-8157 (Print)
ISSN-L
1661-8157
Statut éditorial
Publié
Date de publication
08/2018
Peer-reviewed
Oui
Volume
107
Numéro
16
Pages
902-911
Langue
français
Notes
Publication types: English Abstract ; Journal Article
Publication Status: ppublish
Résumé
Hemophagocytic Lymphohistiocytosis Abstract. Hemophagocytic lymphohistiocytosis (HLH) is a group of rare diseases characterized by over-activation of the immune system. They form two groups: primary and secondary HLH. Primary HLH are linked to mutations impairing lymphocyte cytotoxicity. Secondary HLH are triggered by infections, autoimmune diseases or neoplasia, the remaining cases being labeled idiopathic. HLH manifest as febrile states, cytopenias and hepatosplenomegaly. In the absence of treatment, they quickly lead to multiple organ failure. The diagnosis is currently based on the presence of several clinical and biological markers. Treatment consists of suppression of the triggering factor, organ support and immunosuppression. Primary forms, affecting a pediatric population, have been the subject of intense research, and are nowadays treated with established therapeutic protocols. Several recent retrospective studies have improved our knowledge of secondary HLH, which affects mostly adults and whose incidence seems to be increasing. Thus, new diagnostic criteria are currently being studied for secondary HLH, and several treatment protocols have just been published or are being evaluated.
Mots-clé
Hemophagocytosis, Hämophagozytose, Hémophagocytose, Lymphohistozytose, Makrophagenaktivierungssyndrom, lymphohistiocytose, lymphohistiocytosis, macrophage activation syndrome, syndrome d’activation macrophagique
Pubmed
Création de la notice
14/08/2018 15:09
Dernière modification de la notice
20/08/2019 16:54
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