29-Nov-2019 19:46

CD244 is expressed on all NK cells, gd T cells, and monocytes, on 30%–70% of resting CD8 T cells, and on up to 5% 64 • JID 200 (1 July) • Williams et al. The in vitro triggering of CD244 on NK cells results in increased cytotoxicity and cytokine secretion, including IFN-g [25].This role has been demonstrated in vivo by the excessive production of IFN-g by CD4 and CD8 T cells after lymphocytic choriomeningitis virus infection in SAP-deficient mice [27, 28], and this may provide important clues about the molecular mechanisms of immune activation in IM.Fatal IM is generally associated with the inherited Xlinked lymphoproliferative syndrome (XLP) [12].Primary EBV infection in affected male infants results in T Cell Activation in Mononucleosis • JID 200 (1 July) • 63 Table 1.


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and down-regulation of oncogenes promoting cell cycle arrest and apoptosis. IM is believed to be an immunopathological disease, and it is characterized by dramatic antigen-driven clonal expansions of CD8 T lymphocytes in response to primary Received 27 August 2003; accepted 11 December 2003; electronically published 8 June 2004. After primary infection, the virus establishes life-long persistence in host B lymphocytes [7].

Defined as housebound at the worst point during illness. of copies of Epstein-Barr virus/106 peripheral blood mononuclear cells at the time of diagnosis.

overwhelming cytotoxic T cell activation and death either from fulminating IM with multiorgan failure or EBV-driven lymphoma [13].

The abnormal gene product, signaling lymphocyte activation molecule (SLAM)–associated protein (SAP) [14] or src homology-2 (SH2D1A) [15–16], is a small intracytoplasmic protein with an SH2 domain and is most abundantly expressed in T lymphocytes and NK cells [16–17].


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In vitro, SAP inhibits signal-transduction pathways that are initiated by at least 4 lymphocyte cell-surface receptors: SLAM/CD150, 2B4/CD244, CD84, and LY9 [18–21]. Surface lymphocyte activation marker (SLAM)–associated protein (SAP) regulates lymphocyte activation via signals from cell-surface CD244 (2B4) and SLAM (CD150).

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