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The manifestations of XLP generally occur following Epstein\u2013Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T\u2010cell\u2010mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T\u2010cell populations were observed, but neither demonstrated evidence of lymphoma. Thus, loss of SAP function can lead to dysregulated immune responses characterized by the uncontrolled expansion and activation of T cells independent of EBV infection. Pediatr Blood Cancer 2009;53:1120\u20131123. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.22185","type":"journal-article","created":{"date-parts":[[2009,7,20]],"date-time":"2009-07-20T17:17:52Z","timestamp":1248110272000},"page":"1120-1123","source":"Crossref","is-referenced-by-count":32,"title":["Lymphocytic vasculitis involving the central nervous system occurs in patients with X\u2010linked lymphoproliferative disease in the absence of Epstein\u2013Barr virus infection"],"prefix":"10.1002","volume":"53","author":[{"given":"Kawsar R.","family":"Talaat","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer A.","family":"Rothman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey 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