Dommann S N, Dommann-Scherrer C C, Dours-Zimmermann M T, Zimmermann D R, Kural-Serbes B, Burg G
University Hospital of Zürich, Department of Dermatology, Switzerland.
Arch Dermatol Res. 1996 Apr;288(4):163-7. doi: 10.1007/BF02505218.
Extracutaneous involvement is a sign of poor prognosis in cutaneous T-cell lymphomas (CTCL). Unfortunately it becomes clinically and histologically manifest only late in the course of the disease. It was the purpose of this study to detect clonality in peripheral blood, lymph nodes and bone marrow samples at times when extracutaneous involvement cannot otherwise be demonstrated. In addition to skin biopsies, peripheral blood, lymph node and bone marrow samples from a total of 25 patients were analysed by Southern blotting for clonal gene rearrangement of the T-cell receptor beta-chain. Six of the patients were suffering from mycosis fungoides (MF), four from non-MF CTCL (pleomorphic T-cell lymphomas), seven from Sézary syndrome (SS), eight from pseudolymphoma (insect bites) (PSL), and one from lymphomatoid papulosis (LP). Clonal TcR b gene rearrangements were found in patients with MF in four of five skin probes as well as in two of two lymph node samples and in one of two peripheral blood samples. In SS patients, all skin probes (seven of seven), lymph node samples (six of six), peripheral blood samples (six of six) and one bone marrow specimen had a clonal TcR beta gene rearrangement. In patients with non-MF CTCL, two of four skin, zero of two peripheral blood and one of one bone marrow samples with clonal T cells were detected. All investigated patients showed exactly the same rearrangement pattern at extranodal sites and in the skin, which is proof for the same clone in all compartments. In contrast, no rearrangements were detected in LP and PSL (zero of eight skin probes, zero of two peripheral blood samples). Our results provide strong evidence for an early systemic spread of neoplastic cells in CTCL. However, an initial tumour burden has to be reached in order to lead to a clinically and prognostically relevant manifestation.
皮肤外受累是皮肤T细胞淋巴瘤（CTCL）预后不良的标志。不幸的是，它仅在疾病病程后期才在临床和组织学上表现出来。本研究的目的是在无法通过其他方式证明存在皮肤外受累时，检测外周血、淋巴结和骨髓样本中的克隆性。除皮肤活检外，对总共25例患者的外周血、淋巴结和骨髓样本进行Southern印迹分析，以检测T细胞受体β链的克隆性基因重排。其中6例患者患有蕈样肉芽肿（MF），4例患有非MF型CTCL（多形性T细胞淋巴瘤），7例患有Sezary综合征（SS），8例患有假性淋巴瘤（昆虫叮咬）（PSL），1例患有淋巴瘤样丘疹病（LP）。在MF患者中，5个皮肤样本中的4个、2个淋巴结样本中的2个以及2个外周血样本中的1个检测到克隆性TcR b基因重排。在SS患者中，所有皮肤样本（7个样本中的7个）、淋巴结样本（6个样本中的6个）、外周血样本（6个样本中的6个）和1个骨髓样本均检测到克隆性TcRβ基因重排。在非MF型CTCL患者中，4个皮肤样本中的2个、2个外周血样本中的0个以及1个骨髓样本中的1个检测到克隆性T细胞。所有研究患者在结外部位和皮肤中均显示出完全相同的重排模式，这证明所有区域均为同一克隆。相比之下，LP和PSL患者未检测到重排（皮肤样本8个中的0个，外周血样本2个中的0个）。我们的结果为CTCL中肿瘤细胞的早期全身播散提供了有力证据。然而，必须达到初始肿瘤负荷才能导致临床和预后相关的表现。
