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
in Spanish
| 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
Full text:
Available
Index:
LILACS (Americas)
Main subject:
X Chromosome
/
Glycoproteins
/
T-Lymphocytes, Cytotoxic
/
Carrier Proteins
/
Epstein-Barr Virus Infections
/
Lymphoproliferative Disorders
Limits:
Humans
Language:
Spanish
Journal:
Medicina (B.Aires)
Journal subject:
Medicine
Year:
2003
Type:
Article
Affiliation country:
Argentina
Institution/Affiliation country:
Academia Nacional de Medicina/AR
/
Hospital Británico/AR
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