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德国的皮肤淋巴瘤:德国皮肤病学会(DDG)中央皮肤淋巴瘤登记处的分析。

Cutaneous lymphomas in Germany: an analysis of the Central Cutaneous Lymphoma Registry of the German Society of Dermatology (DDG).

作者信息

Assaf Chalid, Gellrich Sylke, Steinhoff Matthias, Nashan Dorothée, Weisse Franca, Dippel Edgar, Coors Esther, Stein Anette, Gollin Philippa, Henke Ulrike, Adam-Murati Sabrina, Koch André, Klemke Claus-Detlev, Stadler Rudolf, Sterry Wolfram

机构信息

Department of Dermatology, Allergy and Venereology, Skin Tumor Center Charité (HTCC), Charité University Medicine, Berlin, Germany.

出版信息

J Dtsch Dermatol Ges. 2007 Aug;5(8):662-8. doi: 10.1111/j.1610-0387.2007.06337.x.

Abstract

BACKGROUND

Primary cutaneous lymphomas form a heterogeneous group of lymphatic neoplasias. They manifest themselves on the skin and are the second most frequent group of non-Hodgkin lymphoma (NHL) following gastrointestinal lymphomas. The number of epidemiologic studies is small due to limited availability and limited comparability on population-based data.

PATIENTS AND METHODS

In the present study the first evaluation of the German Central Registry for Cutaneous Lymphomas (ZRKL) of the German Society of Dermatology (DDG) is undertaken on the basis of 998 patients. The epidemiology of cutaneous lymphomas in Germany is compared to other national or regional lymphoma registries.

RESULTS

Based on the registration of 998 patients from 26 clinics in Germany,a clear predominance of cutaneous T-cell lymphomas (85 %) in comparison to cutaneous B-cell lymphomas (14 %) is seen. The most frequent representative of CTCL is mycosis fungoides,composing 62 % of cases with a slight predominance of men (M:F = 1.6:1). Differences are also seen in stage of the disease at first presentation of patients with cutaneous lymphomas.While, for example, 80 % of patients with mycosis fungoides in Germany present in early stages (I-IIA),in the USA 34 % of patients are in the tumor stage or have organ involvement at presentation.

CONCLUSIONS

The ZRKL of the DDG for the first time presents epidemiologic data from Germany, allowing comparison with other nations for the study of etio-logical factors and socioeconomic influences. Further, the ZRKL supports the development of uniform and quality-oriented diagnostic criteria and therapeutic options. Finally, the ZRKL provides a foundation for future intensive study of clinical and scientific questions regarding cutaneous T- and B-cell lymphomas.

摘要

背景

原发性皮肤淋巴瘤是一组异质性的淋巴系统肿瘤。它们在皮肤上表现出来,是继胃肠道淋巴瘤之后第二常见的非霍奇金淋巴瘤(NHL)类型。由于基于人群的数据可得性有限且可比性差,流行病学研究的数量较少。

患者与方法

在本研究中,基于998例患者对德国皮肤病学会(DDG)的德国皮肤淋巴瘤中央登记处(ZRKL)进行了首次评估。将德国皮肤淋巴瘤的流行病学与其他国家或地区的淋巴瘤登记处进行了比较。

结果

基于对德国26家诊所998例患者的登记,与皮肤B细胞淋巴瘤(14%)相比,皮肤T细胞淋巴瘤(85%)明显占优势。蕈样肉芽肿是蕈样霉菌病最常见的代表,占病例的62%,男性略占优势(男:女 = 1.6:1)。皮肤淋巴瘤患者首次就诊时的疾病分期也存在差异。例如,德国80%的蕈样肉芽肿患者在早期(I-IIA期)就诊,而在美国,34%的患者在就诊时处于肿瘤期或有器官受累。

结论

DDG的ZRKL首次提供了来自德国的流行病学数据,有助于与其他国家进行比较,以研究病因因素和社会经济影响。此外,ZRKL支持制定统一的、以质量为导向的诊断标准和治疗方案。最后,ZRKL为未来深入研究皮肤T细胞和B细胞淋巴瘤的临床和科学问题奠定了基础。

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