Polgárová Kamila, Polívka Jindřich, Kodet Ondřej, Klener Pavel, Trněný Marek
First Dept. of Internal Medicine - Hematology, University General Hospital in Prague and First Faculty of Medicine, Charles University, Prague, Czechia.
Institute of Hematology and Blood Transfusion, Prague, Czechia.
Front Oncol. 2022 Jun 7;12:884091. doi: 10.3389/fonc.2022.884091. eCollection 2022.
Cutaneous T-cell lymphomas (CTCL) represent rare non-Hodgkin lymphomas (NHL) with an incidence less than 1 per 100,000 inhabitants. The most common type of CTCL is mycosis fungoides (MF), which represents approximately 60% of all CTCL, followed by Sézary syndrome (SS), approximately 5%. We retrospectively analyzed the outcome of 118 patients with MF (n=96) and SS (n=22) treated between the years 1998 and 2021 at the Charles University General Hospital in Prague, Czech Republic. The ratio between men and women was 1.2:1 (62 men, and 56 women). The median age at diagnosis was 62 years (23 to 92 years). From the MF cohort 48 patients (50% out of MF cohort) presented with advanced stage disease. Ninety patients (77%) received a systemic treatment at any time from the diagnosis; the median number of therapy lines was two. At the time of database lock, the overall survival (OS) of 96 patients with MF reached 17.7 years with the median follow-up 4.0 years. With the median follow-up 2.6 years, the median OS of 22 patients with SS was 3.5 years. The most common type of systemic therapy for MF included low-dose methotrexate (61%), interferon-alpha (58%), bexarotene (28%), and chlorambucil (25%). The most common type of therapy for SS included bexarotene (64%), extracorporeal photopheresis (50%), and interferon-alpha (45%). Only the minority of patients received innovative targeted agents including brentuximab vedotin, mogamulizumab, or pembrolizumab. Besides the retrospective analysis of the CTCL cohort, current standards and future perspectives of selected innovative agents are summarized and discussed. The analyzed cohort represents the largest cohort of CTCL patients in the Czech Republic. Overall, the survival parameters of our CTCL cohort are comparable to those previously published by other groups. In conclusion, our analysis of 118 real world cohort of consecutive CTCL patients treated at the single center confirmed the efficacy of immune response modifiers and underlines the urgent need for ample implementation of innovative agents and their combinations into earlier lines of therapy.
皮肤T细胞淋巴瘤(CTCL)是一种罕见的非霍奇金淋巴瘤(NHL),发病率低于十万分之一。CTCL最常见的类型是蕈样肉芽肿(MF),约占所有CTCL的60%,其次是塞扎里综合征(SS),约占5%。我们回顾性分析了1998年至2021年期间在捷克共和国布拉格查理大学总医院接受治疗的118例MF(n = 96)和SS(n = 22)患者的治疗结果。男女比例为1.2:1(62名男性和56名女性)。诊断时的中位年龄为62岁(23至92岁)。MF队列中有48例患者(占MF队列的50%)表现为晚期疾病。90例患者(77%)从诊断开始的任何时间接受了全身治疗;治疗线数的中位数为2。在数据库锁定时,96例MF患者的总生存期(OS)达到17.7年,中位随访时间为4.0年。中位随访2.6年时,22例SS患者的中位OS为3.5年。MF最常见的全身治疗类型包括低剂量甲氨蝶呤(61%)、α干扰素(58%)、贝沙罗汀(28%)和苯丁酸氮芥(25%)。SS最常见的治疗类型包括贝沙罗汀(64%)、体外光化学疗法(50%)和α干扰素(45%)。只有少数患者接受了包括brentuximab vedotin、莫格利珠单抗或帕博利珠单抗在内的创新靶向药物。除了对CTCL队列进行回顾性分析外,还总结并讨论了所选创新药物的当前标准和未来前景。分析的队列代表了捷克共和国最大的CTCL患者队列。总体而言,我们CTCL队列的生存参数与其他组先前发表的参数相当。总之,我们对单中心连续治疗的118例真实世界CTCL患者队列的分析证实了免疫反应调节剂的疗效,并强调迫切需要将创新药物及其联合方案充分应用于早期治疗。