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

@article{Talaat2009LymphocyticVI,
  title={Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection.},
  author={Kawsar Rasmy Talaat and Jennifer A. Rothman and Jeffrey I. Cohen and Mariarita R Santi and John Kim Choi and Miguel Garcia Guzman and Robert Paul Zimmerman and Sudha Nallasamy and Alexander J. Brucker and Martha Quezado and Stefania Pittaluga and Nicholas J. Patronas and Amy D Klion and Kim E Nichols},
  journal={Pediatric blood & cancer},
  year={2009},
  volume={53 6},
  pages={
          1120-3
        }
}
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… CONTINUE READING
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