皮下脂膜炎样T细胞淋巴瘤六例临床及病理特点分析

发布时间：2018-04-24 22:44

本文选题：皮下脂膜炎样T细胞淋巴瘤 + 诊断　；参考：《中华肿瘤防治杂志》2016年23期

【摘要】：目的皮下脂膜炎样T细胞淋巴瘤(subcutaneous panniculitis-like T cell lymphoma,SPTCL)是一类非常罕见的皮肤T细胞恶性淋巴瘤,相关报道较少且易误诊。本研究旨在分析SPTCL患者的临床及病理特征。方法回顾性分析2010-01-01-2015-12-31天津医科大学肿瘤医院收治的6例SPTCL患者的临床治疗、预后及病理特征。结果 6例SPTCL患者中,男3例,女3例。6例患者的年龄2~53岁,平均诊断年龄为29岁,其中4例的患者30岁。起病部位主要为四肢,5例患者表现为皮下的硬结肿物,1例患者表现为皮肤溃疡和渗出。皮肤损害出现的中位时间为13个月(1~60个月)。6例患者中无伴发嗜血细胞综合症(hemophagocytic syndrome,HPS)者。6例患者镜下共同特征是小或中等大小的不典型肿瘤细胞围绕脂肪细胞生长。免疫组化特征为CD3+(100%)、CD4+(100%)、CD8+(100%)、CD20-(100%)、CD56-(83%)、TCRβF-1+(100%)、TIA-1+(100%)、GrB+(100%)、穿孔素阳性(100%)。6例SPTCL患者均接受了以CHOP或CHOP样方案为主的多药联合化疗,化疗后3例患者获得CR,2例患者PR,1例患者SD。CR的3例患者中有2例患者出现复发,其中1例患者经自体造血干细胞移植后获得CR,1例在间断化疗状态下维持SD。获得PR的1例患者经自体造血干细胞移植后获得CR。中位随访时间为47个月(7~73个月),1例失访,3例患者无病生存,2例患者带瘤生存。结论本研究SPTCL患者呈惰性进展且预后较好,常用的化疗方案为CHOP或CHOP样方案,早期复发或化疗效果欠佳的患者采取自体造血干细胞移植可能获益。
[Abstract]:Objective subcutaneous panniculitis-like T cell lymphomatous T cell lymphoma (SPTCLs) is a rare malignant cutaneous T cell lymphoma. The aim of this study was to analyze the clinical and pathological features of SPTCL patients. Methods the clinical treatment, prognosis and pathological features of 6 SPTCL patients admitted to Tianjin Medical University Oncology Hospital from January to January 2015-12-31, 2010-01-01-2015-12-31 were retrospectively analyzed. Results among the 6 patients with SPTCL, there were 3 males and 3 females aged 2 to 53 years, with an average diagnostic age of 29 years, of which 4 cases were 30 years old. The main site of the disease was 5 patients with subcutaneous scleroma and 1 patient with skin ulcer and exudation. The median time of occurrence of skin lesions was from 13 months to 60 months. Among the 6 patients without hemophagocytic syndromes (HPSs), the common feature under microscope was that small or medium size atypical tumor cells grew around adipocytes. Immunohistochemical features were as follows: CD3 TCR 尾 F-1 100 and TIA-1 100. All of the 100 cases of SPTCL were treated with multidrug combination chemotherapy with CHOP or CHOP as the main regimen, and the positive rate of perforin was 100%, and the CD20 ~ (-1) and CD56 ~ (- 1) ~ 83C ~ (3) were used in all the patients with SPTCL, and the TCR 尾 F ~ (-1) was 100% and TIA-1 was 100 ~ (100) and the perforin positive group was 100 ~ (th) ~ (th) ~ (th) ~ (th) th e of the patients with SPTCL. Among the 3 patients with SD.CR, 2 patients had relapse after chemotherapy, and one patient received autologous hematopoietic stem cell transplantation and one patient had maintained SDD after intermittent chemotherapy. One patient with PR obtained CRR after autologous hematopoietic stem cell transplantation. The median follow-up time was 47 months, 7 ~ 73 months, 1 case of lost visit, 3 cases of disease-free survival and 2 cases of tumor-bearing survival. Conclusion in this study, the patients with SPTCL were inert and had a good prognosis. The commonly used chemotherapy regimen was CHOP or CHOP like regimen. Autologous hematopoietic stem cell transplantation may be beneficial to the patients with early recurrence or poor chemotherapeutic effect.
【作者单位】：天津医科大学肿瘤医院血液科天津市肿瘤防治重点实验室国家肿瘤临床医学研究中心天津市恶性肿瘤临床医学研究中心;
【分类号】：R739.5

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