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
Artigo
em Espanhol
| LILACS
| ID: lil-334553
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:
DisponíveL
Índice:
LILACS (Américas)
Assunto principal:
Cromossomo X
/
Glicoproteínas
/
Linfócitos T Citotóxicos
/
Proteínas de Transporte
/
Infecções por Vírus Epstein-Barr
/
Transtornos Linfoproliferativos
Limite:
Humanos
Idioma:
Espanhol
Revista:
Medicina (B.Aires)
Assunto da revista:
Medicina
Ano de publicação:
2003
Tipo de documento:
Artigo
País de afiliação:
Argentina
Instituição/País de afiliação:
Academia Nacional de Medicina/AR
/
Hospital Británico/AR
Similares
MEDLINE
...
LILACS
LIS