摘要
目的:结合文献重点介绍皮下脂膜炎样T细胞淋巴瘤(SPTCL)的临床、病理特点提高病理诊断SPTCL的水平。方法:收集3例皮下脂膜炎样T细胞淋巴瘤进行临床、病理及免疫组化分析,总结经验。结果:3例患者中,男性2例,女性1例,年龄为5岁、26岁、42岁,病变分别位于胸壁、背部四肢及乳腺,呈单发或多发性皮肤红斑及皮下结节,病理观察,由大、中、小异型淋巴细胞浸润皮下脂肪,呈脂膜炎样分布,伴有血管炎,凝固性坏死,吞噬细胞反应等。其中1例噬血细胞现象。3例瘤细胞均表达CD45RO,不表达CD20,3例噬细胞均表达CD68,均行局部皮损切除术,1例失访,余2例病程进展缓慢,缺乏系统症状,分别随访3年及8个月,均存活。结论:SPTCL是由肿瘤性T细胞浸润皮下脂肪形成的一种特殊类型皮肤淋巴瘤,其预后应与肿瘤性T细胞的克隆性有关。
Objective: To explore the clinical presentations and pathologic features of the subcutaneous panniculitis-like T cell lymphoma and to review the literature in order to improve its diagnosis. Methods: Three cases of subcutaneous panniculitis-like T cell lymphoma were collected, and the clinical, pathologic, and immunohistochemical features were analysed. Results: Two cases of male and l case of female patient were included. The age of the patients ranged from 5 to 42. The lesions were located on chest wall, back, limbs and breast, etc. They were presented with single or multiple cutaneous erythematous lesion and subcutaneous nodules. The pathologic features may consist of infiltrations of atypical small, intermediate and large sized lymph cells, infiltrated subcutaneous lipid tissue as panniculitis pattern with vasculitis, coagulation necrosis, and phagocytic reaction. Of them, one case of hemophagocytic phenomenon were present. CD45RO were expressed in the tumor cells of all 3 cases. CD 20 was not expressed. CD68 was expressed in the phagocytes of all 3 cases. Resections of the local lesions were performed in all 3 cases. Loss of the follow-up was in 1 case, the progression of diseases was slow. There was a lack of systemic symptom, and the follow-up visit was 3 years and 8 months, respectively. Conclusion: This is an unique type of cutaneous lymphoma consisted of infiltrations of tumorous T cells into subcutaneous fat. Its prognosis should be associated with the clonality of T cells.
出处
《中国肿瘤临床》
CAS
CSCD
北大核心
2006年第9期510-514,共5页
Chinese Journal of Clinical Oncology
关键词
皮下脂膜炎样T细胞淋巴瘤
皮肤淋巴瘤
噬红细胞综合征
Subcutaneous Panniculitis-like T Cell lymphoma Cutaneous Lymphoma Hemophagocytic syndrome