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

Kawsar R. Talaat; Jennifer A. Rothman; Jeffrey I. Cohen; Mariarita Santi; John K. Choi; Miguel Guzman; Robert Zimmerman; Sudha Nallasamy; Alexander Brucker; Martha Quezado; Stefania Pittaluga; Nicholas J. Patronas; Amy D. Klion; Kim E. Nichols

doi:10.1002/pbc.22185

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

Kawsar R. Talaat, Jennifer A. Rothman, Jeffrey I. Cohen, Mariarita Santi, John K. Choi, Miguel Guzman, Robert Zimmerman, Sudha Nallasamy, Alexander Brucker, Martha Quezado, Stefania Pittaluga, Nicholas J. Patronas, Amy D. Klion, Kim E. Nichols

Bloomberg School of Public Health

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26 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	1120-1123
Number of pages	4
Journal	Pediatric Blood and Cancer
Volume	53
Issue number	6
DOIs	https://doi.org/10.1002/pbc.22185
State	Published - 2009

Keywords

Central nervous system
Vasculitis
X-linked lymphoproliferative disease

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

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10.1002/pbc.22185

Cite this

Talaat, K. R., Rothman, J. A., Cohen, J. I., Santi, M., Choi, J. K., Guzman, M., Zimmerman, R., Nallasamy, S., Brucker, A., Quezado, M., Pittaluga, S., Patronas, N. J., Klion, A. D., & 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. Pediatric Blood and Cancer, 53(6), 1120-1123. https://doi.org/10.1002/pbc.22185

Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection. / Talaat, Kawsar R.; Rothman, Jennifer A.; Cohen, Jeffrey I. et al.
In: Pediatric Blood and Cancer, Vol. 53, No. 6, 2009, p. 1120-1123.

Research output: Contribution to journal › Article › peer-review

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 2009, 'Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection', Pediatric Blood and Cancer, vol. 53, no. 6, pp. 1120-1123. https://doi.org/10.1002/pbc.22185

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

abstract = "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.",

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AU - Rothman, Jennifer A.

AU - Cohen, Jeffrey I.

AU - Santi, Mariarita

AU - Choi, John K.

AU - Guzman, Miguel

AU - Zimmerman, Robert

AU - Nallasamy, Sudha

AU - Brucker, Alexander

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AU - Nichols, Kim E.

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AB - 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.

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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

Abstract

Keywords

ASJC Scopus subject areas

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