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The cytoplasmic protein encoded by the SH2D1A gene plays an essential role in controlling EBV infection. It is expressed in T and NK cells, but not in B cells or in granulocytes. SH2D1A is expressed at a high level in thymus and lung, with a lower level of expression in spleen and liver. Defects in SH2D1A are a cause of X-linked lymphoproliferative disease (XLPD)also known as Duncan disease. XLPD is characterized by an immunodeficiency following Epstein-Barr virus (EBV)infection. Symptoms include fatal mononucleosis, hypogammaglobulinemia, pancytopenia or malignant lymphoma.