Departments of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.
The Japanese Study Group for Cutaneous Lymphomas, The Japanese Skin Cancer Society, Kumamoto, Japan.
J Dermatol. 2018 Jan;45(1):58-63. doi: 10.1111/1346-8138.14100. Epub 2017 Oct 25.
Between 2008 and 2015, 462 newly-diagnosed adult T-cell leukemia/lymphoma (ATLL) patients with cutaneous involvement were found from the nationwide registry for Japanese patients with cutaneous lymphoma, of which 391 were selected for the study. They ranged in age from 28 to 93 years (median, 69 years), and included 215 men and 176 women (male : female ratio = 1.2). The 391 patients comprised 193 (50%) with smoldering type, 52 (13%) with chronic type, 44 (11%) with lymphoma type and 102 (26%) with acute type. The total number of patients in Kyushu/Okinawa was 8.8-times higher than that in Kanto, which was set as the reference value, while the estimated prevalence of human T-lymphotropic virus 1 (HTLV-1) carriers in Kyushu/Okinawa has been reported to be only 2.5-times higher than that in Kanto. In this study, the annual incidence of ATLL per 100 000 residents in Kyushu/Okinawa was 32-times higher than that in Kanto. Our results indicated the higher incidence rate of ATLL in the endemic area than those in the non-endemic areas in Japan, compared with the regional differences of HTLV-1 prevalence determined by serological HTLV-1 screening for blood donors. In addition, this analysis revealed that regional differences of mycosis fungoides/Sézary syndrome incidence rates were very small compared with those of ATLL.
在 2008 年至 2015 年间，从日本皮肤淋巴瘤全国登记处发现了 462 例新发成人 T 细胞白血病/淋巴瘤（ATLL）伴有皮肤受累的患者，其中 391 例入选本研究。这些患者的年龄为 28 岁至 93 岁（中位数为 69 岁），包括 215 名男性和 176 名女性（男：女比例=1.2）。391 例患者中，惰性型 193 例（50%），慢性型 52 例（13%），淋巴瘤型 44 例（11%），急性型 102 例（26%）。九州/冲绳的患者总数是被设为参照值的关东地区的 8.8 倍，而九州/冲绳的人类 T 淋巴细胞病毒 1（HTLV-1）携带者的估计流行率仅比关东地区高 2.5 倍。在本研究中，九州/冲绳的 ATLL 年发病率是关东地区的 32 倍。与血液供者血清学 HTLV-1 筛查确定的 HTLV-1 流行率的区域差异相比，我们的结果表明，ATLL 在流行地区的发病率高于非流行地区。此外，本分析显示蕈样肉芽肿/赛泽里综合征发病率的区域差异与 ATLL 的差异相比非常小。
