Sindrome linfoproliferativo ligado al cromosoma X, infeccion por el virus EBV y defectos en la regulacion de la citotoxicidad linfocitaria / X-linked lymphoproliferative syndrome, EBV virus infection and defects in cytotoxicity lymphocyte regulation
Medicina (B.Aires)
; 63(1): 70-76, 2003. ilus
Article
en Es
| LILACS
| ID: lil-334553
Biblioteca responsable:
BR1.1
ABSTRACT
Mutations in SH2D1A, a gene that codifies for the regulatory protein SAP, result in uncontrolled activation of the SLAM (signaling lymphocyte-activation molecule) pathway. This X-linked immunodeficiency becomes evident when the patients are infected with Epstein Barr virus (EBV) and develop a fulminant form of infectious mononucleosis leading to a lymphoproliferative syndrome that is often fatal (X-linked lymphoproliferative syndrome, XLP). In those who survive, hypogammaglobulinemia and oncohematologic diseases are frequently observed. In this revision, the immuno-regulatory mechanisms involved in XLP immunopathology and the role of different effector cells (CD8 T lymphocytes, NK cells) are discussed
Texto completo:
1
Índice:
LILACS
Asunto principal:
Cromosoma X
/
Glicoproteínas
/
Linfocitos T Citotóxicos
/
Proteínas Portadoras
/
Infecciones por Virus de Epstein-Barr
/
Trastornos Linfoproliferativos
Límite:
Humans
Idioma:
Es
Revista:
Medicina (B.Aires)
Asunto de la revista:
MEDICINA
Año:
2003
Tipo del documento:
Article