Paget样网状细胞增生症临床病理观察  

Pagetoid reticulosis:a clinical and pathologic analysis

作　　者：魏淑飞 徐晓 汪亮亮 黄文勇 张小容 曹芳 李婷 

机构地区：[1]九江学院附属医院病理科,江西九江332000

出　　处：《诊断病理学杂志》2018年第2期126-128,共3页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨Paget样网状细胞增生症(PR)的临床特点、组织学形态特征、免疫组化表型、诊断与鉴别诊断。方法回顾性分析1例Paget样网状细胞增生症的临床病理特征,并对相关文献进行复习。结果病变表面角化过度伴角化不全,棘皮层不规则增厚,上皮脚下延,表皮内及真皮浅层见多量不典型中等大小的淋巴细胞浸润,胞质丰富、淡染,嗜酸性,细胞核可见折叠,呈脑回状,部分可见核仁,细胞核周可见空晕,表皮基底部局灶多个不典型细胞聚集呈巢团状,甚似Pautrier微脓肿,基底膜完整,真皮浅层血管周围淋巴组织浸润,可见个别细胞不典型增生,免疫组化:CD2、CD5、CD3、CD20、CD30、CD4(+),CD8散在(+),Ki-67增殖指数>50%,CD7、TIA-1、Gr B、CD56、TCRαβ、ALK、EMA和TCRγδ(-)。结论 PR是蕈样霉菌病变异型之一,是具有明显亲表皮特征的T细胞淋巴瘤。Objective To analyze the clinical and pathological characteristics,diagnosis and differential diagnosis of disseminated pagetoid reticulosis（ PR）. Methods One case of PR was collected,the clinical and pathological features were investigated with review of the related literatures. Results The lesion was characterized by over hyperplasia of epidermis with hyperkeratosis,parakeratosis and acanthosis. The surface epithelium was infiltrated by medium-sized atypical lymphocytes with abundant and pale eosinophilic cytoplasm,gyriform nuclei,partially visible nucleoli; they were dispersed throughout the epidermis,singly,two to three cells or in small nests. The basement membrane was integritied,and the superficial corium contained a large number of mature lymphocytes and some atypical lymphocytes around the blood vessel. Tumor cells were positive for CD2,CD5,CD3,CD20,CD30,CD4,focally positive CD8,and negative for CD7,TIA-1,Gr B,CD56,TCRαβ,ALK,EMA,and TCRγδ. Ki-67 proliferative index was over 50%. Conclusion PR is a rare variant of mycosis fungoides and indolent cutaneous T-cell lymphoma.

关 键 词：Paget样网状细胞增生症 蕈样霉菌病 组织学特征 

分 类 号：R733[医药卫生—肿瘤]