Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection

Academic Article


  • X-linked lymphoproliferative disease (XLP) is an immunodeficiency caused by defects in the adaptor molecule SAP. The manifestations of XLP generally occur following Epstein-Barr virus (EBV) infection and include fulminant mononucleosis, hypogammaglobulinemia and lymphoma. In this report, we describe two unrelated patients with fatal T-cell-mediated central nervous system vasculitis for whom repeated serologic and molecular testing for EBV was negative. In both patients, clonal T-cell 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. © 2009 Wiley-Liss, Inc.
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    Author List

  • Talaat KR; Rothman JA; Cohen JI; Santi M; Choi JK; Guzman M; Zimmerman R; Nallasamy S; Brucker A; Quezado M
  • Start Page

  • 1120
  • End Page

  • 1123
  • Volume

  • 53
  • Issue

  • 6