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一名具有异常表型的大颗粒淋巴细胞增多症和多态性T细胞受体β链基因重排的患者。

A patient with large granular lymphocytosis of unusual phenotype and polymorphic T-cell receptor beta-chain gene rearrangement.

作者信息

Lin C K, Liu H W, Tse P W, Lai C L, Chan G T

机构信息

Department of Pathology, University of Hong Kong.

出版信息

Am J Clin Pathol. 1990 Aug;94(2):211-6. doi: 10.1093/ajcp/94.2.211.

DOI:10.1093/ajcp/94.2.211
PMID:1695480
Abstract

The authors describe a patient with large granular lymphocytosis who presented with fever of unknown origin and jaundice. Immunophenotyping showed that most of the large granular lymphocytes (LGLs) were CD3-, CD16-, and NKH-1 (Leu-19)+ lymphocytes. Lymphocytosis of this subset of LGLs has not been reported. Analysis of T-cell receptor gene showed polymorphic T-cell receptor beta-chain (TCR beta) gene configuration. Functional studies showed reduced natural killer cell function. The clinical course was very aggressive and resistant to chemotherapy. These features again raise the controversial issues of the ontogeny and heterogeneity of LGLs and their relationship to natural killer cells and T-cells.

摘要

作者描述了一名患有大颗粒淋巴细胞增多症的患者,该患者表现为不明原因发热和黄疸。免疫表型分析显示,大多数大颗粒淋巴细胞(LGL)为CD3-、CD16-和NKH-1(Leu-19)+淋巴细胞。尚未有关于这一LGL亚群淋巴细胞增多症的报道。T细胞受体基因分析显示T细胞受体β链(TCRβ)基因构型呈多态性。功能研究显示自然杀伤细胞功能降低。临床病程进展迅速且对化疗耐药。这些特征再次引发了关于LGL的个体发生、异质性及其与自然杀伤细胞和T细胞关系的争议问题。

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A patient with large granular lymphocytosis of unusual phenotype and polymorphic T-cell receptor beta-chain gene rearrangement.一名具有异常表型的大颗粒淋巴细胞增多症和多态性T细胞受体β链基因重排的患者。
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引用本文的文献

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Aggressive natural killer cell leukemia: report of a Chinese series and review of the literature.侵袭性自然杀伤细胞白血病:一组中国病例报告及文献复习
Int J Hematol. 2007 Jan;85(1):18-25. doi: 10.1532/IJH97.A10612.