蕈样霉菌病24例临床病理

蕈样霉菌病(mycosis fungoides，MF)是一种低度恶性的皮肤T细胞淋巴瘤(cutaneous T cell lymphoma，CTCL),是CTCL中最常见的类型，约占所有皮肤淋巴瘤的50%[1]。经典的MF临床特征主要表现为逐步演变的皮肤斑片、斑块和肿瘤；组织学上显示，具有中等大小脑回状细胞核的异型T淋巴细胞浸润于表皮、真皮及皮下脂肪组织。但由于MF病程进展缓慢，早期临床及病理学表现缺乏特异性，同时MF包含多种亚型和变异型，易误诊为其他疾病。为提高对MF的认识及诊断准确率，回顾性分析本院24例MF患者，探讨其临床病理特点、鉴别诊断、治疗及预后。

资料与方法

一、临床资料

选取2008年至2011年上海交通大学附属瑞金医院皮肤科住院治疗者，并取皮肤活检、经临床及组织病理学确诊且有完整病案资料的24例MF 及其亚型患者，包括23例经典型MF，1例亲毛囊型

·论著·

蕈样霉菌病24例临床病理分析

费晓春，许海敏，吴华成，金晓龙

（上海交通大学医学院附属瑞金医院病理科，上海200025）

[摘要]目的：探讨蕈样霉菌病（MF）的临床病理特点、鉴别诊断、治疗及预后。方法：分析24例MF（包括6例斑片期、11例斑块期和7例肿瘤期）患者的临床资料、病理形态、免疫组化染色、治疗方案及预后。结果：24例MF患者中，男女比为1.7∶1，平均年龄为52岁。临床表现多数符合经典的斑片、斑块至肿瘤期的演变过程。病理表现为真皮内具有脑回状细胞核的异型T淋巴细胞呈灶性、片状或弥漫性浸润，可累及皮下脂肪组织,可见亲表皮性及Pautrier微脓肿。免疫组化肿瘤细胞表达CD3、CD45RO、CD4。早期患者经皮肤靶向治疗及生物学治疗，病情可部分缓解，2例晚期患者给予联合化疗。随访患者中仅有2例在7个月内死亡（平均随访时间1.9年）。结论：MF是皮肤T细胞淋巴瘤中最常见的类型，患者临床表现多样，诊断主要依赖临床特点、病理组织学和免疫组化检查结果。

关键词：蕈样霉菌病；亲表皮性；Pautrier微脓肿；T细胞淋巴瘤；诊断

中图分类号：R733.1文献标识码：A文章编号：1671-2870（2012）04-0361-05

DOI:10.3969/j.issn.1671-2870.2012.04.09

Cutaneous mycosis fungoides：clinicopathological analysis of24cases FEI Xiao-chun，XU Hai-min，WU Hua-cheng，JIN Xiao-long.Department of Pathology,Ruijin Hospital,Shanghai Jiaotong University School of Medicine,Shanghai200025, China

[Abstract]Objective To study the clinicopathological feature，differential diagnosis，therapeutic regimen and prognosis of cutaneous mycosis fungoides(MF).Methods Twenty-four cases of MF were collected,six were in patch stage,eleven in plaque stage and seven in tumor stage.Clinical data,pathologic feature,immunohistochemistry finding, therapeutic regimen,and prognosis were analyzed.Results Of the24cases,ratio of male to female was1.7∶1and mean age was52.Most patients had their skin lesion typically evolved from patch,plaque to tumor.The pathological manifestations were focal,laminar or diffuse infiltration of atypical lymphocytes with cerebriform nuclei presented in dermis,subcutaneous adipose tissue might also be involved.Epidermotropic or Pautrier's microabscess was the typical histological feature in MF.Immunohistochemically,tumor cells were positive for CD3，CD45RO and CD4.Targeted therapy and biologic therapy in early stage patients resulted in partial remission;two advanced cases were treated with combined chemotherapy.During follow-up,2patients died within7months.Conclusions MF,which is the most common type of cutaneous T cell lymphoma,has various clinical presentations.Diagnosis of MF depends on clinical feature,and histopathological and immunohistochemical findings.

Key words:Mycosis fungoides；Epidermotropism；Pautrier's microabscess；T cell lymphoma；Diagnosis

基金项目：上海交通大学医学院课题（YZ1074）

通讯作者：金晓龙E-mail:xljin8@