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循环性塞扎里细胞的克隆性质。

The clonal nature of circulating Sezary cells.

作者信息

Weinberg J M, Jaworsky C, Benoit B M, Telegan B, Rook A H, Lessin S R

机构信息

Department of Dermatology, University of Pennsylvania, Philadelphia 19104, USA.

出版信息

Blood. 1995 Dec 1;86(11):4257-62.

PMID:7492785
Abstract

To determine if circulating Sezary cells can be classified as reactive or neoplastic based on the ability to detect the presence or absence of clonal T-cell receptor beta chain (TCR-beta) gene rearrangements by Southern blot analysis, we evaluated the peripheral blood of 25 patients: 11 patients with Sezary syndrome (SS), 11 with benign inflammatory dermatoses (BID), and three normal controls. Three of 11 patients with SS, with Sezary counts ranging from 14% to 52%, did not demonstrate any clonal TCR-beta gene rearrangements in the peripheral blood, despite a TCR-beta rearrangement by Southern blot analysis in the skin. Ten of 11 BID patients and all normal controls showed no evidence of a TCR-beta gene rearrangement in the peripheral blood. However, one patient with psoriasis demonstrated a TCR-beta gene rearrangement in the peripheral blood. The TCR-beta gene rearrangement detected in this patient, confirmed with polymerase chain reaction (PCR) amplification of the TCR-gamma gene rearrangement, did not correlate with the presence of circulating Sezary cells or the increased risk of neoplasia. Our results indicate that circulating Sezary cells may be monoclonal (neoplastic) or polyclonal (reactive), as defined by TCR gene rearrangement studies. Circulating Sezary cells in SS may be reactive in nature and not accurately reflect the actual tumor burden in the peripheral blood. The presence of circulating Sezary cells or the presence of a clone of cells defined by TCR-beta gene rearrangement in the peripheral blood is not limited to neoplastic disease processes.

摘要

为了根据通过Southern印迹分析检测克隆性T细胞受体β链(TCR-β)基因重排的有无能力,来确定循环中的Sezary细胞能否被分类为反应性或肿瘤性,我们评估了25例患者的外周血:11例Sezary综合征(SS)患者、11例良性炎症性皮肤病(BID)患者和3名正常对照。11例SS患者中有3例,Sezary细胞计数范围为14%至52%,尽管皮肤中通过Southern印迹分析检测到TCR-β重排,但外周血中未显示任何克隆性TCR-β基因重排。11例BID患者中有10例以及所有正常对照在外周血中均未显示TCR-β基因重排的证据。然而,1例银屑病患者在外周血中显示出TCR-β基因重排。该患者中检测到的TCR-β基因重排,经TCR-γ基因重排的聚合酶链反应(PCR)扩增证实,与循环Sezary细胞的存在或肿瘤形成风险增加无关。我们的结果表明,根据TCR基因重排研究的定义,循环中的Sezary细胞可能是单克隆(肿瘤性)或多克隆(反应性)的。SS中的循环Sezary细胞在本质上可能是反应性的,不能准确反映外周血中的实际肿瘤负荷。外周血中循环Sezary细胞的存在或由TCR-β基因重排定义的细胞克隆的存在并不局限于肿瘤性疾病过程。

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Blood. 1995 Dec 1;86(11):4257-62.
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Single TCR-Vβ2 evaluation discloses the circulating T cell clone in Sezary syndrome: one family fits all!单一TCR-Vβ2评估揭示蕈样肉芽肿综合征中的循环T细胞克隆:一种模式适用于所有情况!
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Detection of clonal T-cell receptor gamma gene rearrangements with the use of the polymerase chain reaction in cutaneous lesions of mycosis fungoides and Sézary syndrome.利用聚合酶链反应检测蕈样肉芽肿和塞扎里综合征皮肤病变中的克隆性T细胞受体γ基因重排。
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