Bernengo M G, Novelli M, Quaglino P, Lisa F, De Matteis A, Savoia P, Cappello N, Fierro M T
Department of Medical and Surgical Specialities, First Dermatologic Clinic, University of Turin, Via Cherasco 23, 10126 Torino, Italy.
Br J Dermatol. 2001 Jan;144(1):125-35. doi: 10.1046/j.1365-2133.2001.04014.x.
The lack of specific markers for the phenotyping of circulating neoplastic T cells in Sézary syndrome (SS) patients makes it difficult both to ascertain the presence of clonal cells and to quantify the tumour burden in the peripheral blood. In previous reports we showed that the lack of CD26 (dipeptidyl-aminopeptidase IV) is a characteristic feature of circulating Sézary cells (SC).
The purpose of this study was to ascertain, by means of high-resolution two-, three- or four-parameter flow cytometry, the relationship between CD26 expression on peripheral blood lymphocytes and peripheral blood involvement in cutaneous T-cell lymphoma patients and to assess its significance in SS diagnosis.
The patient population included 52 SS patients, 151 mycosis fungoides (MF) patients at different clinical stages (including 14 with blood involvement, B1-MF), 88 patients with erythrodermic inflammatory skin diseases (EISD) and 72 healthy donors (HD). CD26+ values were available in all cases, whereas CD4+ CD26- level measurement was performed in 23 SS, 141 MF, 71 EISD and 72 HD.
CD4+ CD26- percentage values were higher than 30% in all but one B1-MF and higher than 40% in all SS cases, whereas HD, EISD and B0-MF patient values were always lower than 30%. A statistically significant difference was found in both CD26- and CD4+ CD26- percentage and absolute values between SS and HD, EISD and B0-MF patients. The CD26- and CD4+ CD26- percentage values (but not the absolute values) were significantly higher in B1-MF compared with HD, EISD and B0-MF patients (P < 0.001). Moreover, CD26- absolute values and CD4+ CD26- percentage and absolute values were significantly higher in SS than in B1-MF (P < 0.001). A statistically significant direct relationship was found between CD4+ CD26- percentage values and the percentage of circulating SC within the lymphoid population in SS and B1-MF (r = 0.77; P < 0.001). The lack of CD26 was confirmed on phenotypically clonal cells in patients with an expanded circulating TCRvbeta population or a T-cell antigen loss. Sorted CD4+ CD26- cells from both SS patients and HD showed the characteristic cerebriform nuclei of SC.
We feel that a CD4+ CD26- percentage value higher than 30% of peripheral blood lymphocytes could correctly identify the presence of peripheral blood involvement in SS and MF patients.
缺乏用于对 Sézary 综合征（SS）患者循环肿瘤性 T 细胞进行表型分析的特异性标志物，这使得确定克隆细胞的存在以及定量外周血中的肿瘤负荷都变得困难。在先前的报告中，我们表明 CD26（二肽基氨基肽酶 IV）的缺失是循环 Sézary 细胞（SC）的一个特征性特征。
本研究的目的是通过高分辨率的两参数、三参数或四参数流式细胞术，确定外周血淋巴细胞上 CD26 表达与皮肤 T 细胞淋巴瘤患者外周血受累情况之间的关系，并评估其在 SS 诊断中的意义。
患者群体包括 52 例 SS 患者、151 例处于不同临床阶段的蕈样肉芽肿（MF）患者（包括 14 例有血液受累的，B1 - MF）、88 例红皮病型炎症性皮肤病（EISD）患者和 72 名健康供者（HD）。所有病例均有 CD26 + 值，而在 23 例 SS、141 例 MF、71 例 EISD 和 72 名 HD 中进行了 CD4 + CD26 - 水平测量。
除 1 例 B1 - MF 外，所有患者的 CD4 + CD26 - 百分比值均高于 30%，所有 SS 病例均高于 40%，而 HD、EISD 和 B0 - MF 患者的值始终低于 30%。在 SS 与 HD、EISD 和 B0 - MF 患者之间，CD26 - 和 CD4 + CD26 - 的百分比及绝对值均存在统计学显著差异。与 HD、EISD 和 B0 - MF 患者相比，B1 - MF 患者的 CD26 - 和 CD4 + CD26 - 百分比值（但绝对值无此差异）显著更高（P < 0.001）。此外，SS 患者的 CD26 - 绝对值以及 CD4 + CD26 - 的百分比和绝对值均显著高于 B1 - MF 患者（P < 0.001）。在 SS 和 B1 - MF 患者中，发现 CD4 + CD26 - 百分比值与淋巴样群体中循环 SC 的百分比之间存在统计学显著的直接关系（r = 0.77；P < 0.001）。在循环 TCRvbeta 群体扩大或 T 细胞抗原丢失的患者中，表型克隆细胞上 CD26 的缺失得到证实。从 SS 患者和 HD 中分选的 CD4 + CD26 - 细胞显示出 SC 的特征性脑回状核。
我们认为外周血淋巴细胞中 CD4 + CD26 - 百分比值高于 30%能够正确识别 SS 和 MF 患者外周血受累的存在。
