狼疮性脂膜炎和皮下脂膜炎样T细胞淋巴瘤的临床对比研究  

Clinical Comparative Analysis of Lupus Panniculitis and Subcutaneous Panniculitis-like T-cell Lymphoma

作　　者：温蓬飞[1] 周沁田 万川 刘晓羽[4] 解瑶[1] 李凡[1] 王琳[1] WEN Peng-fei;ZHOU Qin-tian;WAN Chuan;LIU Xiao-yu;XIE Yao;LI Fan;WANG Lin(Department of Dermatology and Venereology,West China Hospital,Sichuan University,Chengdu 610041,China;Department of Dermatology,the First Affiliated Hospital of PLA General Hospital,Chengdu 610041,China;Department of Dermatology,Dermatology Hospital of Jiangxi Province,Nanchang 330001,China;Department of Pathology,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院皮肤性病科,成都610041 [2]解放军总医院第一附属医院皮肤科,北京100048 [3]江西省皮肤病专科医院皮肤科,南昌330001 [4]四川大学华西医院病理科,成都610041

出　　处：《四川大学学报（医学版）》2019年第6期930-934,共5页Journal of Sichuan University(Medical Sciences)

摘　　要：目的分析狼疮性脂膜炎(LEP)和皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床及病理特征。方法从临床病理、免疫表型和T细胞受体(TCR)基因重排等方面对9例狼疮性脂膜炎(LEP)和9例皮下脂膜炎样T细胞淋巴瘤(SPTL)进行比较研究。结果①临床特点:LEP好发于头面部,通常无系统症状,对羟氯喹治疗反应较好,预后好;SPTL更易累及下肢,可伴系统症状,若肿瘤播散或合并噬血细胞综合征(HPS)可能提示预后不良。②组织病理:LEP以淋巴细胞为主的浸润常累及真表皮,可见少量的浆细胞;SPTL几乎不累及真表皮,可见瘤细胞的"花环样"浸润模式和"豆袋细胞"。③免疫表型:LEP组CD4^+/CD8^+、团块状CD20^+、较多CD138^+和散在CD123^+;SPTL组CD4^-/CD8^+、βF1^+,CD138^-、CD123^-,TIA-1^+或GrB^+更提示SPTL。④TCR-γ基因重排:LEP(-)而SPTL(+)。结论大多数LEP和SPTL各具临床病理特征,易于区分;病变局限于皮下脂肪层的LEP需借助免疫组化标记及基因重排检测等协助鉴别。Objective To explore the similarities and differences in clinical pathological features and gene rearrangement of lupus erythematosus profundus(LEP) and subcutaneous panniculitis-like T-cell lymphoma(SPTL). Methods We compared the clinical presentations, histopathology, immunophenotypical features and T-cell receptor(TCR) gene rearrangement findings of 9 cases of LEP and SPTL. Results For clinical features, most patients of LEP occurred on head and face without systemic symptoms. LEP patients responded well to hydroxycholorquine treatment with good prognosis. Most patients of SPTL tended to lower extremities involvement and accompanied with systemic symptoms, the patients with disseminated lesions or hemophagocytic syndrome(HPS) showed poorer prognosis. For histopathology, LEP patients showed dense inflammatory infiltrate in the dermis consisting predominantly of lymphocytes with less numbers of plasma cells. However, the dermis was spared in SPTL, and rimming of adipocytes and erythrophagocytosis was observed in SPTL. Lymphocytes of LEP expressing CD4^+/CD8^+, as well as clusters of CD20^+. CD138-positive cells and scatter of CD123-positive cells were also observed in LEP. Tumor cells of SPTL were CD4^-/CD8^+, βF1^+, CD138^- and CD123^-. The expression of TIA-1 or GrB was more favor in SPTL. Monoclonal T-cell receptor-γ gene rearrangement was found in 89% of SPTL patients while negative for LEP. Conclusion Base on different clinical and pathological features, it is easy to distinguish LEP from SPTL. However, a minority of lesions in LEP localize at subcutaneous tissue, which may turn to immunophenotypical and TCR gene rearrangement test for diagnosis.

关 键 词：狼疮性脂膜炎 深在性红斑狼疮 皮下脂膜炎样T细胞淋巴瘤 

分 类 号：R73[医药卫生—肿瘤]