IRCCS Azienda Ospedaliero-Universitaria di Bologna, Dermatology Unit, Italia, Department of medicine and surgery (DIMEC), University of Bologna, Bologna, Italy.
AOU delle Marche, Hematology Department, Ancona, Italy, Department of Clinical and Molecular Science (DISCLIMO), Università Politecnica delle Marche, Ancona, Italy.
Eur J Dermatol. 2023 Aug 1;33(4):360-367. doi: 10.1684/ejd.2023.4531.
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.
在原发性皮肤 T 细胞淋巴瘤（CTCL）中，蕈样真菌病（MF）是最常见的，与 Sezary 综合征（SS）一起，也是研究最多的亚型。大多数关于 MF 和 SS 流行病学的研究都是基于小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，但大多数研究都存在局限性，如选择偏差以及缺乏临床信息或随访数据。因此，无法得出可靠的结论。本文综述了目前我们对 MF 和 SS 流行病学的理解所依据的数据，并根据从意大利马尔凯地区皮肤淋巴瘤登记处检索到的数据提出了一些原创发现。马尔凯地区皮肤淋巴瘤登记处是一个多学科团队，成立于 27 年前，旨在共同管理这些罕见疾病。所有经临床和组织学确诊为原发性皮肤淋巴瘤的患者都集中在意大利安科纳的马尔凯理工大学血液病诊所进行临床评估、分期、治疗和随访。本文强调了意大利建立皮肤原发性 T 细胞淋巴瘤全国登记处的必要性，因为该国除了马尔凯地区皮肤淋巴瘤登记处外，没有详细的流行病学信息。全国登记处可以从意大利各地更全面地收集数据，并能提供更准确的发病率和流行病学信息。这将有助于了解这些疾病的发病机制和诊断程序，并改善患者的预后。因此，我们建议在意大利建立皮肤原发性 T 细胞淋巴瘤全国登记处。
