Good Syndrome: an adult onset immune deficiency remarkable for its high incidence of invasive infections and autoimmune complications.
Fiche publication
Date publication
mars 2015
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BONNOTTE Bernard
Tous les auteurs :
Malphettes M, Gerard L, Galicier L, Boutboul D, Asli B, Szalat R, Perlat A, Masseau A, Schleinitz N, Le Guenno G, Viallard JF, Bonnotte B, Thiercelin-Legrand MF, Sanhes L, Borie R, Georgin-Lavialle S, Fieschi C, Oksenhendler E
Lien Pubmed
Résumé
BACKGROUND: Good Syndrome (GS) is a rare condition in which thymoma is associated with hypogammaglobulinemia. It is characterized by increased susceptibility to bacterial, viral and fungal infections, as well as autoimmunity. Most patients have no circulating B cells. METHODS: The French DEFI cohort provides detailed clinical and immunological descriptions of 690 adults with primary hypogammaglobulinemia. Comparisons between GS, common variable immune deficiency disorders (CVID) and B(-) CVID (B cells
Référence
Clin Infect Dis. 2015 Mar 31. pii: civ269.