Abstract：
Among primary cutaneous T-cell lymphomas (CTCL), mycosis fungoides (MF) is the most frequent and, along with Sézary syndrome (SS), the best-studied subtype. Most available studies on epidemiology of MF and SS are based on small cohorts or different inclusion criteria. Moreover, although this has become a hot topic, most studies show limitations, such as selection bias and lack of clinical information or follow-up data. Therefore, no reliable conclusions can be drawn. This paper reviews the current data underpinning our understanding of the epidemiology of MF and SS, and presents some original findings based on data retrieved from the cutaneous lymphoma registry of the Italian Marche region. The Marche Regional Cutaneous Lymphoma Registry is a multidisciplinary team founded 27 years ago to share the management of these rare disorders. All patients with a clinical and histologically confirmed diagnosis of primary cutaneous lymphoma are centralized in Ancona (Italy) at the Haematology Clinic, Polytechnic University of Marche, for clinical evaluation, staging, treatment, and follow-up. This paper emphasizes the need for a national registry of pCLs in Italy, as no detailed epidemiological information is available in the country except for the Marche Regional Cutaneous Lymphoma Registry. A national registry would allow for more comprehensive data collection from all over Italy and could provide more accurate information on incidence and epidemiology. This would be beneficial for understanding the pathogenesis and diagnostic procedures of these diseases and could improve patient outcomes. Therefore, we advise the creation of a national registry of pCLs in Italy.
摘要：
在原发性皮肤T细胞淋巴瘤（CTCL）中，霉菌病（MF）是最常见的，连同Sézary综合征（SS），研究最好的亚型。大多数关于MF和SS流行病学的现有研究都是基于小型队列或不同的纳入标准。此外，尽管这已经成为一个热门话题，大多数研究都显示出局限性，如选择偏倚和缺乏临床信息或随访数据。因此,无法得出可靠的结论。本文回顾了支持我们对MF和SS流行病学理解的当前数据，并根据从意大利马尔凯地区皮肤淋巴瘤登记处检索的数据提出了一些原始发现。Marche区域皮肤淋巴瘤登记处是一个27年前成立的多学科团队，旨在分享这些罕见疾病的管理。所有经临床和组织学证实诊断为原发性皮肤淋巴瘤的患者都集中在安科纳（意大利）的血液学诊所，马尔凯理工大学,用于临床评估，分期,治疗,和后续行动。本文强调了在意大利建立pCLs国家注册的必要性，由于除马尔凯地区皮肤淋巴瘤登记处外，该国没有详细的流行病学信息。国家登记册将允许从意大利各地收集更全面的数据，并可以提供关于发病率和流行病学的更准确信息。这将有利于理解这些疾病的发病机理和诊断程序，并可以改善患者的预后。因此,我们建议在意大利建立一个国家的pCLs注册。
