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慢性粒细胞白血病急变期的免疫球蛋白和T细胞受体基因重排

Immunoglobulin and T-cell receptor gene rearrangement in blast crisis of chronic myelocytic leukemia.

作者信息

Miller W J, Gonzalez-Sarmiento R, Kersey J H

机构信息

Department of Medicine, University of Minnesota Hospitals, Minneapolis 55455.

出版信息

Exp Hematol. 1988 Nov;16(10):884-8.

PMID:3139437
Abstract

Chronic myelocytic leukemia (CML) displays a wide repertoire in its terminal phase, with blast cells showing characteristics of myeloid, B-lymphoid, or T-lymphoid cells in some patients. Blast crisis (BC) cells from 14 patients were studied for immunoglobulin (Ig)- and T-cell-associated gene rearrangements. Five myeloid BC patients had no Ig- or T-cell-associated gene rearrangement. In contrast, all eight patients with pure lymphoid BC displayed C mu rearrangement and two also showed kappa-light chain rearrangement. One patient with mixed (lymphoid and erythroid) BC, however, showed neither Ig- nor T-cell-associated rearrangements. One patient displayed both Ig- (C mu) and T-cell-associated (T beta and T gamma) rearrangements. These cells expressed CD9, CD10, and CD24 surface antigens, but no T-cell antigens. Although most lymphoid blast crises appear to represent an early stage in B-cell differentiation, some cells have undergone apparently inappropriate gene rearrangements during differentiation. Such cells may have been immortalized while undergoing normally occurring nonproductive rearrangement or may, due to their malignant nature, display abnormal genotypic characteristics.

摘要

慢性粒细胞白血病(CML)在终末期表现出广泛的谱系,在一些患者中,原始细胞表现出髓系、B淋巴细胞系或T淋巴细胞系细胞的特征。对14例患者的急变期(BC)细胞进行了免疫球蛋白(Ig)和T细胞相关基因重排的研究。5例髓系BC患者未发生Ig或T细胞相关基因重排。相比之下,所有8例纯淋巴细胞性BC患者均出现Cμ重排,2例还出现κ轻链重排。然而,1例混合性(淋巴细胞性和红系)BC患者既未出现Ig也未出现T细胞相关重排。1例患者同时出现Ig(Cμ)和T细胞相关(Tβ和Tγ)重排。这些细胞表达CD9、CD10和CD24表面抗原,但不表达T细胞抗原。尽管大多数淋巴细胞性急变期似乎代表B细胞分化的早期阶段,但一些细胞在分化过程中发生了明显不适当的基因重排。这些细胞可能在进行正常发生的无效重排时被永生化,或者由于其恶性性质而表现出异常的基因型特征。

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