Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection.
Lymphocytic vasculitis involving the central nervous system occurs in patients with X-linked lymphoproliferative disease in the absence of Epstein-Barr virus infection.
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DOI：
10.1002/pbc.22185
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发表时间：
2009-12
影响因子：
3.2
通讯作者：
Nichols, Kim E.
中科院分区：
文献类型：
--
作者：
Talaat, Kawsar R.;Rothman, Jennifer A.;Cohen, Jeffrey I.;Santi, Mariarita;Choi, John K.;Guzman, Miguel;Zimmerman, Robert;Nallasamy, Sudha;Brucker, Alexander;Quezado, Martha;Pittaluga, Stefania;Patronas, Nicholas J.;Klion, Amy D.;Nichols, Kim E.
X连锁淋巴组织增生性疾病（XLP）是一种由衔接分子SAP缺陷引起的免疫缺陷。XLP的表现通常发生在EB病毒（EBV）感染后，包括暴发性单核细胞增多症、低丙种球蛋白血症和淋巴瘤。在这份报告中，我们描述了两个不相关的致命性T细胞介导的中枢神经系统血管炎的患者，他们反复进行EBV的血清学和分子检测均为阴性。在这两名患者中，均观察到克隆T细胞群，但均未表现出淋巴瘤的证据。因此，SAP功能的丧失可导致免疫应答失调，其特征在于不依赖于EBV感染的T细胞的不受控制的扩增和活化。
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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