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具有大颗粒淋巴细胞形态的扩增Tγ细胞群体——I. 免疫学、临床及形态学特征

Expanded T gamma cell populations with the morphology of large granular lymphocytes -- I. Immunological, clinical and morphological characterization.

作者信息

Herrmann F, Sieber G, Komischke B, Schreckenberg A, Ludwig W D, Rühl H

出版信息

Leuk Res. 1983;7(5):667-80. doi: 10.1016/0145-2126(83)90137-6.

Abstract

Lymphocytes from two patients with T gamma cell proliferations displaying the morphology of large granular lymphocytes (LGL) were characterized in terms of cell marker phenotyping and immunologic functions. In both patients, the lymphocytes were positive for E-R, HuTLA, OKT5, OKT8, OKT11, OKM1, VEP13, Leu1, Leu2a, Lyt2 and Lyt3 and were negative for Tmu, Tar, SIg, BA1, BA2, EM-R, C3d-R, C3b-R, OKT6, OKT9, Leu3a, OKIa1 and TdT. In addition, investigations for T411, T811 and M522 in patient 1 yielded positive results. There were differences in the phenotype of the two patients with regard to the reactions with OKT3, OKT10 and VEP10. While, in patient 1, OKT3 was very pronounced and OKT10 and VEP10 were completely negative, OKT10 and VEP10 were very pronounced in patient 2, whereas OKT3 was positive only in a very small percentage of cells. Though the lymphocytes in both patients were potent effectors of NK and K functions (patient 2 more strongly than patient 1) and a noticeably reduced mitogen response was shown to PHA, Con A and zinc, patient 1 showed a distinct suppression of allogenic and autologous B cell response to transformation into ISC, coinciding with the clinical observation of a hypogammaglobulinemia; neither B cell suppression nor dysgammaglobulinemia was seen in patient 2. The results are discussed with regard to other comparable T gamma proliferations reported in the literature.

摘要

对两名患有呈现大颗粒淋巴细胞(LGL)形态的Tγ细胞增殖症患者的淋巴细胞,从细胞标志物表型分析和免疫功能方面进行了特征鉴定。在两名患者中,淋巴细胞E-R、HuTLA、OKT5、OKT8、OKT11、OKM1、VEP13、Leu1、Leu2a、Lyt2和Lyt3呈阳性,而Tmu、Tar、SIg、BA1、BA2、EM-R、C3d-R、C3b-R、OKT6、OKT9、Leu3a、OKIa1和TdT呈阴性。此外,对患者1的T411、T811和M522检测结果呈阳性。两名患者在与OKT3、OKT10和VEP10反应的表型上存在差异。在患者1中,OKT3反应非常明显,OKT10和VEP10完全阴性;而在患者2中,OKT10和VEP10反应非常明显,OKT3仅在很小比例的细胞中呈阳性。尽管两名患者的淋巴细胞都是NK和K功能的有效效应细胞(患者2比患者1更强),并且对PHA、Con A和锌的丝裂原反应明显降低,但患者1对同种异体和自体B细胞转化为ISC的反应表现出明显抑制,这与低丙种球蛋白血症的临床观察结果一致;患者2未出现B细胞抑制或球蛋白异常血症。结合文献中报道的其他类似Tγ增殖症对结果进行了讨论。

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