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T细胞淋巴瘤与病毒相关噬血细胞综合征。

T-cell lymphoma and the virus-associated hemophagocytic syndrome.

作者信息

Craig F E, Clare C N, Sklar J L, Banks P M

机构信息

University of Texas Health Science Center, San Antonio.

出版信息

Am J Clin Pathol. 1992 Feb;97(2):189-94. doi: 10.1093/ajcp/97.2.189.

DOI:10.1093/ajcp/97.2.189
PMID:1372145
Abstract

A 20-month-old girl had a disorder that by both clinical and histologic criteria resembled the virus-associated hemophagocytic syndrome in the setting of Epstein-Barr virus infection. Subsequent investigation revealed histologic evidence of disseminated T-cell lymphoma. DNA hybridization studies displayed a monoclonal T-cell receptor beta chain rearrangement, in the absence of clonal immunoglobulin gene rearrangement, and a single band in the analysis for the fused termini of the Epstein-Barr virus genome. These results suggest the presence of a monoclonal population of T lymphocytes infected with Epstein-Barr virus. The diagnosis of lymphoma was confirmed at autopsy. The authors discuss the association of Epstein-Barr virus infection with the development of T-cell lymphoma and propose that the previous reports of virus-associated hemophagocytic syndrome include cases of unrecognized T-cell lymphoma.

摘要

一名20个月大的女童患有一种疾病,根据临床和组织学标准,该疾病在爱泼斯坦-巴尔病毒感染的背景下类似于病毒相关噬血细胞综合征。随后的调查显示有播散性T细胞淋巴瘤的组织学证据。DNA杂交研究显示存在单克隆T细胞受体β链重排,而无克隆性免疫球蛋白基因重排,并且在爱泼斯坦-巴尔病毒基因组融合末端分析中出现一条带。这些结果提示存在感染爱泼斯坦-巴尔病毒的单克隆T淋巴细胞群。尸检证实了淋巴瘤的诊断。作者讨论了爱泼斯坦-巴尔病毒感染与T细胞淋巴瘤发生的关联,并提出先前关于病毒相关噬血细胞综合征的报告中包括了未被识别的T细胞淋巴瘤病例。

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