Biostatistics Unit, Institute for Social and Preventive Medicine, University of Zürich, Zürich, Switzerland.
Br J Dermatol. 2011 May;164(5):1071-7. doi: 10.1111/j.1365-2133.2010.10143.x.
Epidemiological data on primary cutaneous lymphomas (PCLs) are rare and have not previously been investigated in Switzerland.
To analyse variations in demographics, the pattern of subtypes and staging during the two 10-year intervals, 1990-1999 and 2000-2009.
This was a descriptive study of 263 patients with PCL based on a retrospective review and reassessment according to the World Health Organization/European Organization for Research and Treatment of Cancer classification.
Change was observed in the pattern of cutaneous T-cell lymphoma subtypes: the frequency of Sézary syndrome decreased from 17% to 7% and the frequency of CD30+ lymphoproliferative disorders increased from 7% to 18% (overall P = 0·04). Staging of PCL showed a higher number of cases of early-stage mycosis fungoides (P = 0·01). In relation to the international data, the Zürich group had a higher number of patients with Sézary syndrome (11% vs. 3%) and marginal cell lymphoma (14% vs. 5-7%). In addition, comparison of the survival data showed prolonged median overall survival of Zürich patients with Sézary syndrome in the second 10-year interval (6·5 vs. 2-4 years).
The increasing frequency of marginal cell lymphoma and CD30+ lymphoproliferative disorders might depend on an increased awareness of these diseases in the medical community, driven by progress in the classification and staging of these disease entities.
原发性皮肤淋巴瘤（PCL）的流行病学数据很少，以前在瑞士也没有进行过研究。
分析 1990-1999 年和 2000-2009 年两个 10 年期间发病率、亚型和分期的变化。
这是一项基于回顾性分析和根据世界卫生组织/欧洲癌症研究与治疗组织分类重新评估的 263 例 PCL 患者的描述性研究。
观察到皮肤 T 细胞淋巴瘤亚型的模式发生了变化：蕈样肉芽肿的频率从 17%下降到 7%，CD30+淋巴增生性疾病的频率从 7%增加到 18%（总体 P=0·04）。PCL 的分期显示早期蕈样真菌病的病例数增加（P=0·01）。与国际数据相比，苏黎世组的 Sezary 综合征患者（11%比 3%）和边缘细胞淋巴瘤患者（14%比 5-7%）更多。此外，生存数据的比较显示，苏黎世 Sezary 综合征患者在第二个 10 年期间的中位总生存时间延长（6·5 比 2-4 年）。
边缘细胞淋巴瘤和 CD30+淋巴增生性疾病的发病率增加可能与医学界对这些疾病的认识提高有关，这是由于这些疾病实体的分类和分期的进展所推动的。
