皮下脂膜炎样T细胞淋巴瘤六例临床及病理特点分析

Subcutaneous panniculitis-like T-cell lymphoma:A clinical and pathologic study of 6 cases

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。

OBJECTIVE Subcutaneous panniculitis-like T-cell lymphoma（SPTCL）is a rare skin T cell lymphoma with a high misdiagnosis rate and the related research is limited.This study aimd to describe the clinical and pathologic features of SPTCL patients in our medical center.METHODS Six SPTCL patients from January 1,2010 to December31,2015 in Tianjin Medical University Cancer Institute and Hospital were retrospectively reviewed.RESULTS The 6SPTCL patients consisted of 3 males and 3females（male∶female=1∶1）,the median age at diagnosis was 29years（range：2-53years）.Four of 6patients were younger than 30 years.The initial presenting skin lesions involved groin,hip,forearm,thigh,especially limbs.Five patients were demonstrated subcutaneous nodes,one patient behaved cutaneous ulceration and effusion.The duration of skin lesions before diagnosis ranged from 1month to more than 5years（median：13months）.There was no patient with hemophagocytic syndrome.The characters noticed microscopically revealed atypical small-to-medium size cells rimming of adipocytes with irregular nucleus,nuclear fission,karyorrhexis without infiltration of epidermis and dermis.The immunohistochemistry revealed that CD3＋（100%）,CD4＋（100%）,CD8＋（100%）,CD20-（100%）,CD56-（83%）,TCRβF-1＋（100%）,TIA-1＋（100%）,GrB＋（100%）,perforin＋（100%）.All the 6patients received chemotherapy,three patients got CR,two patients received PR and one patient SD（living with residual disease）.Two of the three CR patients relapsed and one patient received CR after auto-hematopoietic stem cell transplantation（auto-HSCT）,the other one patient sustained SD under persistent chemotherapy.One of the two PR patients got CR after the treating of auto-HSCT.The median follow-up time was 47months（from 7to 73months）.Among the 6patients,three sustained CR and two sustained SD at the end of the follow-up period except for one patient losing follow-up.CONCLUSIONS SPTCL has an indolent clinical course with well prognosis.The frequently-used regimen was CHOP or CHOP-like chemotherapy.The relapse/refractory or showing minimal reaction to chemotherapy patients may obtain a benefit from high doses of chemotherapy and autologous stem cell transplant（HD-ASCT）.