Department of Dermatology, Kagoshima University, Kagoshima, Japan.
Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan; Department of Dermatology, Takamatsu Red Cross Hospital, Takamatsu, Japan.
J Dermatol Sci. 2020 Mar;97(3):187-193. doi: 10.1016/j.jdermsci.2020.01.010. Epub 2020 Jan 25.
The types of cutaneous lymphoma (CL) and their incidences can vary among geographic areas or ethnic groups.
This study aimed to investigate the incidence of various CL types in Japan using epidemiological data from a nationwide registration system for CL.
A questionnaire was sent to participating hospitals, all of which had been approved to conduct residency programs for board-certified dermatologists by the Japanese Dermatological Association. Data from patients newly diagnosed with CL were collected electronically.
Between 2012 and 2017, 2547 new patients with CL from the dermatological institutes were registered. In total, 2090 patients had primary CL and 453 had secondary CL. Those with primary CL included 1609 (77.0 %) patients with mature T- and natural killer (NK)-cell neoplasms, 442 (21.1 %) with B-cell neoplasms, and 39 (1.9 %) with blastic plasmacytoid dendritic cell neoplasms. Mycosis fungoides (MF) was the most common CL subtype in the present study (1003 patients, 48.0 %), and 72.4 % of MF patients had early-stage disease, similar to observations in previous studies on other cohorts. Primary cutaneous CD30+ T-cell lymphoproliferative disorders and adult T-cell leukemia/lymphoma were the second and third most common subtypes, respectively.
Compared to that in our previous cohort (2007-2011), the number of registered T- and NK-cell CL cases decreased, whereas that of B-cell CL cases increased from 44.8-73.7 patients/year. These results provide insight into CL trends within the Japanese population, which might contribute to a better understanding of the disease.
皮肤淋巴瘤（CL）的类型及其发病率在不同地理区域或种族群体中可能有所不同。
本研究旨在使用日本 CL 全国登记系统的流行病学数据，调查日本各种 CL 类型的发病率。
向参与的医院发送了一份问卷，所有医院均已获得日本皮肤科协会认证的皮肤科住院医师培训计划的批准。通过电子方式收集新诊断为 CL 的患者的数据。
在 2012 年至 2017 年间，从皮肤科研究所登记了 2547 例新的 CL 患者。共有 2090 例患者患有原发性 CL，453 例患有继发性 CL。原发性 CL 患者中包括 1609 例（77.0%）成熟 T 和自然杀伤（NK）细胞肿瘤患者、442 例（21.1%）B 细胞肿瘤患者和 39 例（1.9%）母细胞性浆细胞样树突细胞肿瘤患者。蕈样真菌病（MF）是本研究中最常见的 CL 亚型（1003 例，48.0%），72.4%的 MF 患者为早期疾病，与其他队列的既往研究观察结果相似。原发性皮肤 CD30+T 细胞淋巴增生性疾病和成人 T 细胞白血病/淋巴瘤分别是第二和第三常见的亚型。
与我们之前的队列（2007-2011 年）相比，登记的 T 和 NK 细胞 CL 病例数量减少，而 B 细胞 CL 病例数量从每年 44.8-73.7 例增加。这些结果提供了日本人群中 CL 趋势的见解，这可能有助于更好地了解该疾病。
