髓系肉瘤伴T细胞异常表达病例的临床特征、形态学、免疫表型及分子遗传学研究

Clinical,Morphological,Immunophenotypic and Genetic Analysis of Myeloid Sarcoma with Abnormal T-cell Expression

目的探讨髓系肉瘤(myeloid sarcoma,MS)伴T细胞异常表达病例的临床病理特征。方法选取陕西省人民医院2021年8月6日确诊的1例髓系肉瘤,采用HE染色、免疫组织化学和原位杂交EBER检测,观察其组织学特征、免疫表型以及原位杂交EBER结果,随访患者,并进行相关文献复习。结果患者女性,52岁,活检部位为右颈部淋巴结,镜下髓系肉瘤肿瘤细胞形态单一,幼稚,染色质细腻,核分裂像易见,散在不成熟嗜酸性粒细胞。免疫组织化学CD43,髓过氧化物酶(myeloperoxidase,MPO),CD56阳性;CD34及CD7部分阳性;CD3,CD4,CD8,CD2,CD5,TdT,CD20,CD79a,CD19,PAX-5,Grb,TIA-1,MUM-1,CD10,ALK,CD117和CD1a阴性,CD21未见FDC网,Ki67指数约60%。原位杂交EBER阴性。结论髓系肉瘤表达T细胞表面标志罕见,诊断需要结合临床、形态、免疫表型及遗传学等综合分析判断,以避免误诊为其它淋巴造血来源肿瘤。

Objective To investigate the clinicopathological features of myeloid sarcoma(MS)with abnormal T cell expression.Methods A case of MS diagnosed in Shaanxi Provincial People’s Hospital on August 6,2021 was studied by HE staining,immunohistochemistry and EBER in situ hybridization,and reviewed related literature.Results The female patient was 52 years old.Biopsy of right cervical lymph nodes was performed that the tumor cells of MS were simple and immature,with fine chromatin,obvious mitotic figures and scattered immature eosinophils.Immunohistochemistry showed that these tumor cells were positive for CD43,myeloperoxidase(MPO)and CD56,with partial expression of CD34 and CD7.These cells did not express CD3,CD4,CD8,CD2,CD5,TdT,CD20,CD79a,CD19,PAX-5,Grb,TIA-1,MUM-1,CD10,ALK,CD117 and CD1a.CD21 showed no FDC network.Ki67 index was about 60%.EBER in situ hybridization was negative.Conclusion Myeloid sarcoma expressing T cell is rare,and the diagnosis should be combined with clinical,morphological,immunophenotypic and genetic analysis to avoid misdiagnosing as other lymphohematopoietic tumors.