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Establishment of Epstein-Barr Virus Growth-transformed Lymphoblastoid Cell Lines
06:38

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Published on: November 8, 2011

[X-linked lymphoproliferative syndrome, EBV virus infection and defects in cytotoxicity lymphocyte regulation]

Alejandro Malbrán1, Liliana Belmonte, Beatriz Ruibal-Ares

  • 1Hospital Británico, Buenos Aires.

Medicina
|April 4, 2003
PubMed

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.

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