皮下脂膜炎性T细胞性淋巴瘤的临床特征及分子生物学特点

Clinical and molecular biological characteristics of subcutaneou panniculitic T-cell lymphoma

目的 :对皮下脂膜炎性T细胞淋巴瘤 (SPTCL)的临床特征、病理、免疫表型及分子生物学特点进行研究。方法 :临床观察及实验室、病理检查研究SPTCL的临床、病理特点 ,免疫表型通过用LCA、CD3、UCHL、L2 6单抗进行石蜡免疫过氧化物酶染色、用αβTCR、γδTCR抗体进行冷冻切片免疫过氧化物酶的染色 ,用识别V1δ、V2δ 的抗体进行γδTCR亚型分析 ,PCR检测SPTCL患者TCR基因重排。结果 :7例SPTCL患者均有皮下结节、高热、体重下降 ,5例出现嗜血细胞综合征 ,7例SPTCL患者均有电介质、酸碱平衡失调及酶学检查的异常。 7例SPTCL患者的病理均在脂肪组织内见大量异型淋巴细胞浸润 ,均表达LCA、CD3、UCHL ,不表达L2 6。 4例SPTCL患者中有 3例表达γδTCR ,1例表达αβTCR ,3例γδTCR患者均表达V2 +δ ,4例有 3例出现TCRγ基因重排。结论 :SPTCL大多病情严重 ,肿瘤细胞来源于T细胞系的皮肤淋巴细胞组织相关的V2 +δ 细胞 ,可以出现TCRγ基因重排。

Objective:To study the clinical pathologic,immunophenotypic and clinical and molecular biological characteristics of subcutaneous panniculitic T-cell lymphoma.Methods:The clinical and pathologic features were studied by clinical observation,laboratory test and clinical pathology.Immunophenotypic features were measured by paraffinnimmunoperoxidase with the antibodies of LCA?CD3?UCHL?L26,by freeze section immunoperoxidase with the antibodies of αβTCR?γδTCR and analysis of the subtype with the antibodies of V 1 δ?V 2 δ.T cell receptor-γ gene rearrangements were studied by polymerase chain reaction(PCR).Results:Cutaneous node,high fever,loss of body weight appeared in seven patients and hemophagocytic syndrome in five patients.Imbalance of dielectric,acid and basic,abnormality of the enzyme involvement of the subcutaneous fat in a lacelike pattern with neoplastic cells were seen in all patients.The expression of LCA?CD 3?UCHL occurred in all patients,but no L26.The expression of γδTCR was found in three patients whose γδTCR was V 2+ δ and the expression of αβTCR was found in one of four patients.T cell receptor-γ gene rearrangements were seen in three patients.Conclusion:SPTCL is a critical disease.The tumor cells origin from V 2+ δ of T-lymphocyte relating to cutaneous lymphotissue.T cell receptor-γ gene rearrangements may happened in SPTCL patient.