皮下脂膜炎样T细胞淋巴瘤九例临床病理及免疫表型研究  

Clinicopathologic analysis and immunophenotype of nine cases of subcutaneous panniculitis-like T- cell lymphoma

作　　者：旷翠娥 李巧飞[2] 曹光玲[3] 韩建德[3] 陈小红[3] Kuang Cui＇ e Li Qiaofei Cao Guangling Han Jiande Chen Xiaohong(Department of Dermatology, Shenzhen Baoan People＇s Hosphal, Shenzhen 518101, China Department of Dermatology, Nanfang Hospital, Southern Medical University, Guangzhou 51051 Department of Dermatology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510080, Chin)

机构地区：[1]深圳宝安区人民医院皮肤科,518101 [2]南方医科大学南方医院皮肤科 [3]中山大学附属第一医院皮肤科

出　　处：《中华皮肤科杂志》2017年第8期553-556,共4页Chinese Journal of Dermatology

摘　　要：目的 分析皮下脂膜炎样T细胞淋巴瘤（SPTL）的临床及组织病理表现、免疫表型、治疗和预后。方法 回顾性分析9例SPTL患者的临床与实验室资料，完善相关病理及免疫组化标记，并随访。结果 9例患者中8例表现为多发皮下结节和肿块，主要累及下肢（8例）和躯干（6例），7例伴发热。3例行全身PET-CT，7例行骨髓穿刺，未见皮肤外内脏系统肿瘤，均不伴噬血细胞综合征。皮肤组织病理示异形单个核细胞核大、深染，主要浸润皮下脂肪组织，围绕单个脂肪细胞呈环状排列，真皮层附属器和血管周围可见肿瘤细胞浸润（5/9例）。免疫表型示9例肿瘤细胞表达βF1、CD3、CD8，8例表达粒酶B和T细胞胞内抗原1（TIA-1），均不表达CD4、CD20、CD30、CD56。5例接受化疗（1例儿童和1例产后妇女），1例儿童接受甲泼尼龙冲击治疗，随访8例治疗后均达到临床完全缓解。结论 SPTL来源于α/β T细胞，组织病理和免疫组化检查有助于诊断及鉴别诊断。Objective To analyze the clinical and histopathological features, immunophenotypes, treatment and prognosis of subcutaneous panniculitis-like T cell lymphoma （SPTL）. Methods Clinical and experimental data were collected from 9 cases of SPTL, and retrospectively analyzed. Related pathological and immunohistochemical markers were examined by Envision method. Eight patients were followed up. Results Of the 9 patients, 8 had multiple subcutaneous nodules and plaques, which mainly involved the lower limbs in 8 patients and the trunk in 6 patients. Seven patients had fever. Three patients were subjected to the whole-body positron emission tomography-computed tomography （PET-CT）, and 7 to bone marrow aspiration. No visceral tumors and hemophagocytic syndrome were found. Histopathological examination of skin lesions showed atypical mononuclear cells with large nuclei and deep staining, which mainly infiltrated the subcutaneous adipose tissue and were arranged in a circular pattern. Among 9 patients, infiltration of tumor cells was observed around skin appendages and blood vessels in the dermis in 5 patients. Immunohistochemical examination showed positive staining for βF1, CD3 and CD8 in tumor cells in 9 cases, positive staining for granzyme B and T-cell-restricted intracellular antigen-1 （TIA-1） in 8 cases, and negative staining for CD4, CD20, CD30 and CD56 in all the patients. Five patients received chemotherapy, including a child and a postpartum woman. One child received methylprednisolone pulse therapy. During the follow-up, 8 patients achieved a complete clinical remission after treatment. Conclusion SPTL is derived from α/β T cells, and histopathological and immunohistochemical examina-tions can be helpful for its diagnosis and differential diagnosis.

关 键 词：淋巴瘤 T细胞 脂膜炎 皮下组织 病理学 临床 免疫组织化学 

分 类 号：R739.5[医药卫生—肿瘤]