Barton A D
Department of Medicine, The Stamford Hospital, Connecticut, USA.
Cancer. 1997 Oct 15;80(8):1409-17. doi: 10.1002/(sici)1097-0142(19971015)80:8<1409::aid-cncr7>3.0.co;2-9.
Peripheral T-cell lymphocytosis is found on very rare occasions in patients with thymomas. The immunophenotypic features and clonality of the lymphocytes in tumor and peripheral blood now are elucidating this enigmatic phenomenon.
The author presents what is believed to be the seventh case of peripheral T-cell lymphocytosis associated with thymoma and reviews the previous six cases. The pathology slides of the thymoma were reviewed with a pathologist who confirmed the presence of neoplastic thymic epithelium with cytokeratin stains. Immunophenotyping by flow cytometry was performed at Dianon Systems, Inc., on both the thymoma cells and cells in the peripheral blood. In addition, gene rearrangement analysis was performed on the peripheral lymphocytes using the previously described Southern blot analysis technique with immunoglobulin probes (heavy chain, kappa light chain, and lambda light chain) and T-cell receptor gene probes (beta and gamma chains).
Analyses of the T cells within the thymoma and the peripheral blood confirm that the peripheral T cells are both polyclonal and more mature than those populating the thymoma. Clearly the peripheral T cells are not themselves neoplastic, and yet they represent more than physical "spillover" of the immature tumor-related T cells.
Peripheral T-cell lymphocytosis occurs rarely with locally aggressive, lymphocytic thymomas. Although it is clear that these cells are not neoplastic, as they are in other T-cell proliferative disorders, the etiology of this unusual phenomenon remains obscure and may reflect the perturbation of systemic immunoregulation that accompanies thymic neoplasia. It is important to differentiate this condition from T-cell lymphomas or leukemia to treat affected patients appropriately.
外周 T 细胞淋巴细胞增多症在胸腺瘤患者中极为罕见。肿瘤及外周血中淋巴细胞的免疫表型特征和克隆性正在阐明这一神秘现象。
作者报告了据信是第七例与胸腺瘤相关的外周 T 细胞淋巴细胞增多症病例,并回顾了此前的六例病例。与病理学家共同复查胸腺瘤的病理切片,病理学家通过细胞角蛋白染色证实了肿瘤性胸腺上皮的存在。在 Dianon 系统公司对胸腺瘤细胞和外周血中的细胞进行了流式细胞术免疫表型分析。此外,使用先前描述的 Southern 印迹分析技术,用免疫球蛋白探针(重链、κ轻链和λ轻链)和 T 细胞受体基因探针(β链和γ链)对外周淋巴细胞进行基因重排分析。
对胸腺瘤和外周血中的 T 细胞分析证实,外周 T 细胞是多克隆的,且比胸腺瘤中的 T 细胞更成熟。显然,外周 T 细胞本身并非肿瘤性的,但它们所代表的不仅仅是不成熟的肿瘤相关 T 细胞的物理“溢出”。
外周 T 细胞淋巴细胞增多症很少发生于局部侵袭性淋巴细胞性胸腺瘤。虽然很明显这些细胞不像其他 T 细胞增殖性疾病中的细胞那样具有肿瘤性,但这种不寻常现象的病因仍然不明,可能反映了胸腺肿瘤伴随的全身免疫调节紊乱。将这种情况与 T 细胞淋巴瘤或白血病区分开来对于恰当治疗受影响的患者很重要。