皮下脂膜炎样T细胞淋巴瘤的免疫表型、基因重排检测及其在诊断中的意义

目的 探讨免疫表型和基因重排检测在皮下脂膜炎样T细胞淋巴瘤(SPTL)的诊断和鉴别诊断中的意义.方法 参照2005年世界卫生组织-欧洲癌症研究与治疗组织(WHO-EORTC)皮肤淋巴瘤分类标准收集20例SPTL.采用10种抗原标记进行免疫表型检测,运用PCR技术检测TCRγ、IgH基因重排,并用EB病毒编码的小RNA1/2(EBER1/2)原位杂交检测EB病毒感染.结果 (1)本组病例中男9例、女11例,平均年龄29.5岁;(2)所有病例的瘤细胞均表达1个或多个T细胞分化抗原(CD2、CD3或CD45RO),18/19病例表达BF1,18/20病例表达CD8,20/20、16/20病例分别表达细胞毒颗粒相关蛋白TIA-1、颗粒酶B,瘤细胞不表达CD4、CD20和CD56;(3)16/20病例检出TcRγ基因重排,未检出IgH基因重排;(4)5/20病例EBERl/2原位杂交阳性.结论 SPTL的瘤细胞具有克隆性TCR基因重排,综合临床病理、免疫表型及基因重排检测有助于本病确诊.

Diagnostic significance of immunophenotyping and detection of gene rearrangement in subcutaneous panniculitis-like T-cell lymphoma

Objective To explore the diagnostic implication of immunophenotyping and gene rearrangement in subcutaneous panniculitis-like T-cell lymphoma(SPTL).Methods According to the selection criteria of 2005 WHO-EORTC classification for cutaneous lymphomas,20 SPIL patients were enrolled in this study.A 10-antibody panel was used for immunophenotyping and in addition,polymerase chain reaction for TCRγ and IgH gene rearrangement and in situ hybridization for EBER1/2 were also employed. Results There were 9 males and 11 female with a mean age of 29.5 years.Immunophenotypic study showed that all the patients expressed one to three T-cell associated antigens(CD2,CD3 or CD45RO),18 patients were positive for BF1,18 for CD8,20 for TIA-1 and 16 for granzyme B.None of the patients expressed CD4,CD20 and CD56.TCRγ gene rearrangement was found in 16 of 20 cases(80.0%)and none for IgH gene rearrangement.The positive rate of EBER1/2 Was 25.0%(5/20).Conclusions Since the majority of SPTL patients show clonal TCR gene rearrangements,correlations among clinical presentation,histological features,immunophenotype and gene rearrangement data are considered important in confirming a diagnosis of SPTL.