Background:Primary cutaneous peripheral T-cell lymphoma, not otherwise specified (pcPTCL-NOS), is a rare and aggressive form of lymphoma. Its characteristics and treatment outcomes remain poorly understood.Methods:We identified 15 patients who were diagnosed with pcPTCL-NOS between January 2014 and August 2024 at Tianjin Medical University Cancer Institute and Hospital (TMUCIH) in this retrospective study. The clinical and immunophenotypic features, treatment regimens, and outcomes of these patients were investigated.Results:All patients (4 men, 11 women; median age 54 years) presented with skin lesions, including five stage T1, four stage T2 and six stage T3 lesions. pcPTCL-NOS manifests clinically either with solitary or disseminated rapidly growing nodules/tumors and papules and, less often, ulcers. The lesion sites in patients presenting with solitary/localized tumors (stage T1 and T2) were the head and limbs, and those in patients presenting with disseminated lesions (stage T3) were the trunk, head, and limbs. The CD4/CD8 immunophenotypic characteristics were as follows: CD4+/CD8− 53.33%; CD4+/CD8+ 26.67%; CD4−/CD8− 13.33%; and CD4−/CD8+ 6.67%. One patient had a T follicular helper (TFH) phenotype. Five patients had aberrant expression of the B-cell marker CD20 by tumor cells. All patients received CHOP or CHOP-like regimens as the initial treatment, with three patients undergoing complete lesion resection before chemotherapy, seven patients receiving treatment combined with chidamide (tucidinostat), two patients receiving treatment combined with brentuximab vedotin, two patients receiving treatment combined with mitoxantrone liposomes (Lipo-Mit), three patients receiving treatment combined with radiotherapy, and two patients receiving ASCT after the first-line treatment. The OS rates at 1 year, 2 years, and 3 years were 80%, 77.8%, and 77.8%, respectively; the PFS rates were 60%, 44.4%, and 33.3%, respectively. With a median follow-up of 40 months, the median PFS was 21 months, and the median OS was not reached. Univariate analyses revealed that patients with B symptoms and the CD4−/CD8− phenotype had inferior outcomes (p< 0.05). Age, sex, tumor stage, PIT score, Ki-67 index, elevated β2-MG levels, expression of CD20 or PD1, and treatment selection were not associated with the prognosis. A trend of a survival benefit in patients with solitary (T1) tumors compared with patients with disseminated (T2, T3) tumors was observed, suggesting that it is possible to reduce the intensity of treatment in patients with T1 tumors in the future.Conclusions:pcPTCL-NOS is an aggressive but poorly characterized lymphoma that may require early and active systemic treatment. However, for patients with T1 tumors, reducing the intensity of treatment with CHOP should be appropriately considered.
背景：原发性皮肤外周T细胞淋巴瘤，非特指型（pcPTCL-NOS）是一种罕见且侵袭性的淋巴瘤。其临床特征和治疗结局目前仍知之甚少。 方法：在这项回顾性研究中，我们纳入了2014年1月至2024年8月期间在天津医科大学肿瘤医院（TMUCIH）确诊的15例pcPTCL-NOS患者。分析了这些患者的临床及免疫表型特征、治疗方案及结局。 结果：所有患者（男性4例，女性11例；中位年龄54岁）均表现为皮肤病变，其中T1期5例，T2期4例，T3期6例。pcPTCL-NOS的临床表现可为孤立性或播散性快速生长的结节/肿块和丘疹，较少表现为溃疡。表现为孤立性/局限性肿块（T1和T2期）的患者，病变部位位于头部和四肢；而表现为播散性病变（T3期）的患者，病变部位位于躯干、头部和四肢。CD4/CD8免疫表型特征如下：CD4+/CD8− 占53.33%；CD4+/CD8+ 占26.67%；CD4−/CD8− 占13.33%；CD4−/CD8+ 占6.67%。1例患者具有T滤泡辅助细胞（TFH）表型。5例患者的肿瘤细胞异常表达B细胞标志物CD20。所有患者初始治疗均接受CHOP或类CHOP方案，其中3例患者在化疗前接受了病灶完全切除，7例患者接受了联合西达本胺的治疗，2例患者接受了联合维布妥昔单抗的治疗，2例患者接受了联合米托蒽醌脂质体（Lipo-Mit）的治疗，3例患者接受了联合放疗的治疗，2例患者在一线治疗后接受了自体干细胞移植（ASCT）。1年、2年和3年的总生存（OS）率分别为80%、77.8%和77.8%；无进展生存（PFS）率分别为60%、44.4%和33.3%。中位随访40个月，中位PFS为21个月，中位OS尚未达到。单因素分析显示，伴有B症状和CD4−/CD8−表型的患者预后较差（p<0.05）。年龄、性别、肿瘤分期、PIT评分、Ki-67指数、β2-微球蛋白（β2-MG）水平升高、CD20或PD1的表达以及治疗方案选择与预后无关。观察到孤立性（T1期）肿瘤患者相较于播散性（T2、T3期）肿瘤患者存在生存获益的趋势，提示未来可能对T1期肿瘤患者降低治疗强度。 结论：pcPTCL-NOS是一种侵袭性强但特征尚不明确的淋巴瘤，可能需要早期积极的全身治疗。然而，对于T1期肿瘤患者，应适当考虑降低CHOP方案的治疗强度。
肿瘤(癌症)患者之家