SciELO - Scientific Electronic Library Online

vol.63 número1EutanasiaRecomendaciones divergentes en el diagnóstico precoz del cáncer de próstata índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Servicios Personalizados



  • No hay articulos citadosCitado por SciELO

Links relacionados

  • No hay articulos similaresSimilares en SciELO


Medicina (Buenos Aires)

versión On-line ISSN 1669-9106


MALBRAN, A. et al. X-linked lymphoproliferative syndrome, EBV infection and impaired regulation of cell-mediated cytotoxicity. Medicina (B. Aires) [online]. 2003, vol.63, n.1, pp. 70-76. ISSN 1669-9106.

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.

Palabras clave : XLP; SAP; SLAM; EBV; NK activity; 2B4.

        · resumen en Español     · texto en Español     · Español ( pdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License