Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81, Irwon-ro, Gangnam-Gu, Seoul, 06351, Korea.
Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Department of Lymphoma, Peking University Cancer Hospital and Institute, Beijing, 100142, China.
Int J Hematol. 2021 Sep;114(3):355-362. doi: 10.1007/s12185-021-03179-7. Epub 2021 Jul 24.
Cutaneous T-cell lymphomas (CTCLs) are a group of T-cell lymphomas with low incidence. Due to their indolent characteristics, treatment strategies have not yet been established for advanced CTCLs. In this study, relative incidence of CTCLs in Asia was estimated and the therapeutic outcomes presented based on various treatments currently used in clinics for advanced CTCLs. As part of a prospective registry study of peripheral T-cell lymphoma (PTCL) conducted across Asia, including Korea, China, Taiwan, Singapore, Malaysia, and Indonesia, subgroup analysis was performed for patients with CTCLs. Among 486 patients with PTCL, 37 with CTCL (7.6%) were identified between April 2016 and February 2019. Primary cutaneous ALK-negative anaplastic large cell lymphoma (ALCL, 35.1%) was the most common subtype. With a median follow-up period of 32.1 months, median progression-free survival (PFS) was 53.5 months (95% CI 0.0-122.5), and overall survival was not reached. 14 patients (48.2%) underwent subsequent treatment after the first relapse, but the response rate was 20% with a PFS of 2.2 months (95% CI 0.3-4.0). Six patients received autologous stem cell transplantation (auto-SCT). However, auto-SCT did not result in better outcomes. Additional studies are needed on standard care treatment of advanced or refractory and relapsed CTCLs.
皮肤 T 细胞淋巴瘤(CTCL)是一组发病率较低的 T 细胞淋巴瘤。由于其惰性特征,目前尚未为晚期 CTCL 制定治疗策略。本研究估计了亚洲 CTCL 的相对发病率,并根据目前临床上用于晚期 CTCL 的各种治疗方法展示了治疗结果。作为在亚洲进行的外周 T 细胞淋巴瘤(PTCL)的前瞻性注册研究的一部分,包括韩国、中国、中国台湾、新加坡、马来西亚和印度尼西亚,对 CTCL 患者进行了亚组分析。在 486 例 PTCL 患者中,2016 年 4 月至 2019 年 2 月期间发现 37 例 CTCL(7.6%)。原发性皮肤间变性大细胞淋巴瘤(ALK-阴性 ALCL,35.1%)是最常见的亚型。中位随访 32.1 个月,中位无进展生存期(PFS)为 53.5 个月(95%CI 0.0-122.5),总生存期未达到。14 例(48.2%)在首次复发后接受了后续治疗,但缓解率为 20%,PFS 为 2.2 个月(95%CI 0.3-4.0)。6 例患者接受了自体干细胞移植(auto-SCT)。然而,auto-SCT 并未带来更好的结果。需要对晚期、难治性和复发性 CTCL 的标准治疗进行更多的研究。