摘要
目的:研究皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床病理特征、免疫表型、组织起源和预后。方法:按照2005年皮肤淋巴瘤世界卫生组织-欧洲癌症治疗研究组织(WHO-EORTC)新分类分析6例SPTL患者的临床资料,作常规组织病理检查和免疫组化标记,并对6例患者石蜡组织和1例患者冰冻新鲜组织切片进行βF1标记。结果:6例患者中男2例,女4例,中位年龄25.5岁。临床皮损以无痛性皮下结节和(或)斑块为主,少数患者有发热、贫血和脾大。瘤细胞主要限于皮下脂肪组织内,有异形性和核分裂,并见不同程度脂肪坏死和组织细胞吞噬现象。瘤细胞表达βF1、CD3、CD8、TIA-1、GB、LCA和CD45RO,不表达CD4、CD30、CD56、CD20和CD79a。6例SPTL患者平均随访37个月内均获缓解,仅1例在确诊42个月后因并发糖尿病、高血压及心衰而死亡。结论:SPTL是一种起源于αβT淋巴细胞的罕见类型细胞毒性皮肤淋巴瘤,临床病程迁延反复,5年生存率高达80%以上。完整充分的免疫组化标记对SPTL的确诊和分型必不可少。
Objective： To investigate the clinicopathologic features, immunophenotype, cell derivation and prognosis of subcutaneous panniculitis-like T-cell lymphoma （SFTL）. Methods： According to the new WHO-EORTC classification for cutaneous lymphomas, clinical data, histological features and immunohistochemistry of six SPTL cases were studied. Six paraffin-embedded specimens and one frozen specimen were marked with betaF1, respectively. Results： Among six patients with median age of 25.5, two were males and four were females. They usually presented with painless nodules and/or plaques. A few had fever, anemia and splenomegaly. Neoplastic cells were mainly limited to the subcutaneous tissue with nuclear atypia and mitosis. Variable fat necrosis and cytophagocytosis were seen. Neoplastic cells were positive for betaF1, CD3, CD8, TIA-1, GB, LCA and CD45RO but negative for CD4, CD30, CD56, CD20 and CD79a. All six patients got complete remission with a median follow-up of 37 months, and only one died of diabetes mellitus, hypertension and heart failure 42 months after the diagnosis. Conclusions： SPTL is a rare cytotoxic T-cell lymphoma derived from alpha/beta T cell, which has a protracted clinical course with a 5-year survival rate of over 80% of patients. Complete immunochemical studies are essential for the correct diagnosis of SPTL.
出处
《临床皮肤科杂志》
CAS
CSCD
北大核心
2008年第8期489-492,共4页
Journal of Clinical Dermatology
