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

Publication ,  Journal Article
Talaat, KR; Rothman, JA; Cohen, JI; Santi, M; Choi, JK; Guzman, M; Zimmerman, R; Nallasamy, S; Brucker, A; Quezado, M; Pittaluga, S; Klion, AD ...
Published in: Pediatr Blood Cancer
December 2009

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.

Duke Scholars

Published In

Pediatr Blood Cancer

DOI

EISSN

1545-5017

Publication Date

December 2009

Volume

53

Issue

6

Start / End Page

1120 / 1123

Location

United States

Related Subject Headings

  • Vasculitis
  • T-Lymphocytes
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Oncology & Carcinogenesis
  • Male
  • Lymphoproliferative Disorders
  • Lymphatic Vessels
  • Intracellular Signaling Peptides and Proteins
  • Humans
  • Genetic Diseases, X-Linked
 

Citation

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Talaat, K. R., Rothman, J. A., Cohen, J. I., Santi, M., Choi, J. K., Guzman, M., … Nichols, K. E. (2009). Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. Pediatr Blood Cancer, 53(6), 1120–1123. https://doi.org/10.1002/pbc.22185
Talaat, Kawsar R., Jennifer A. Rothman, Jeffrey I. Cohen, Mariarita Santi, John K. Choi, Miguel Guzman, Robert Zimmerman, et al. “Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection.Pediatr Blood Cancer 53, no. 6 (December 2009): 1120–23. https://doi.org/10.1002/pbc.22185.
Talaat KR, Rothman JA, Cohen JI, Santi M, Choi JK, Guzman M, et al. Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. Pediatr Blood Cancer. 2009 Dec;53(6):1120–3.
Talaat, Kawsar R., et al. “Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection.Pediatr Blood Cancer, vol. 53, no. 6, Dec. 2009, pp. 1120–23. Pubmed, doi:10.1002/pbc.22185.
Talaat KR, Rothman JA, Cohen JI, Santi M, Choi JK, Guzman M, Zimmerman R, Nallasamy S, Brucker A, Quezado M, Pittaluga S, Patronas NJ, Klion AD, Nichols KE. Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. Pediatr Blood Cancer. 2009 Dec;53(6):1120–1123.
Journal cover image

Published In

Pediatr Blood Cancer

DOI

EISSN

1545-5017

Publication Date

December 2009

Volume

53

Issue

6

Start / End Page

1120 / 1123

Location

United States

Related Subject Headings

  • Vasculitis
  • T-Lymphocytes
  • Signaling Lymphocytic Activation Molecule Associated Protein
  • Oncology & Carcinogenesis
  • Male
  • Lymphoproliferative Disorders
  • Lymphatic Vessels
  • Intracellular Signaling Peptides and Proteins
  • Humans
  • Genetic Diseases, X-Linked