原发性皮肤T细胞淋巴瘤患者的临床特征及预后分析

    Clinical Characteristics and Prognosis of Patients With Primary Cutaneous T-cell Lymphoma

    • 摘要: 目的 探讨原发性皮肤T细胞淋巴瘤(cutaneous T-cell lymphoma,CTCL)的临床特征、治疗方式及预后。 方法 回顾性分析徐州医科大学附属医院2009年11月至2019年5月经皮肤活检确诊为原发性CTCL患者15例,以描述这种罕见肿瘤。 结果 确诊患者中,男性8例,女性7例,中位年龄50岁,绝大多数患者(13/15)因皮肤病变就诊。各亚型的CTCL皮损形态各异。蕈样肉芽肿(mycosis fungoides,MF)主要表现为斑块(83.3%);皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T-cell lymphoma,SPTCL)主要表现为肢体多发性皮下结节(75%);外周T细胞淋巴瘤非特指型(peripheral T-cell lymphoma not otherwise specified,PTCL-NOS)皮损呈多态性;主要皮损部位为肢体(7例)和全身(3例)。各亚型的CTCL临床病理特征不同。瘤细胞表面以T细胞抗原表达为主,Ki-67阳性指数5%~80%,1例EB病毒编码区(Epstein-Barr encoding region,EBER)为(+)。中位随访时间为54.3个月,2年总生存率(overall survival,OS)为66.7%,2年无进展生存率(progression-free survival,PFS)为43.9%。7例死亡患者中位生存时间为18个月(14.0~57.2个月)。惰性或低危组患者的4年OS(P=0.045 4)和PFS(P=0.002 3)显著高于侵袭性或高危组,MF、SPTCL、PTCL-NOS之间的PFS有显著差异(P=0.033 4)。 结论 本研究中,除MF或SPTCL外,其余CTCL亚型侵袭性强,预后差。各亚型皮损表现及组织病理形态多样化,诊断时应结合临床表现、组织病理检查、免疫表型等,以免漏诊、误诊。

       

      Abstract: Objective To investigate the clinical features, treatment, and prognosis of primary cutaneous T-cell lymphoma (CTCL). Methods Totally 15 patients with primary CTCL diagnosed by skin biopsy in the affiliated Hospital of Xuzhou Medical University from November 2009 to May 2019 were retrospectively analyzed to describe this rare tumor. Results The main results were as follows: 8 were males and 7 were females, and the median age was 50 years old. The vast majority of patients (13/15) see a doctor for skin lesions. The skin lesions of different subtypes of CTCL vary in shape. The main manifestation of mycosis fungoides (MF) was plaque (83.3%); Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) was mainly characterized by multiple subcutaneous nodules of the extremities (75%); peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) lesions were polymorphic. The main lesions were limb (n=7) and whole body (n=3). The clinicopathological features of different subtypes of CTCL were different. T cell antigen was mainly expressed on the surface of tumor cells. The positive index of Ki-67 was 5%~80%. Epstein-Barr encoding region (EBER) was positive in only 1 case. The median follow-up time was 54.3 months, 2-year overall survival (OS) was 66.7%, and 2-year progression-free survival (PFS) was 43.9%. The median survival time of 7 dead patients was 18 months (range 14.0~57.2). The 4-year OS (P=0.045 4) and PFS (P=0.002 3) of patients in the indolent or low-risk group were significantly better than those in the aggressive or high-risk group, and there were significant differences in PFS among MF, SPTCL, and PTCL-NOS (P=0.033 4). Conclusions In this study, except for MF or SPTCL, the other CTCL subtype had strong invasiveness and poor prognosis. The manifestations and histopathological morphology of various subtypes of skin lesions were diversified. Clinical manifestations, histopathological examination, and immunophenotype should be combined in the diagnosis to avoid missed diagnosis and misdiagnosis.

       

    /

    返回文章
    返回