摘要目的 总结并探讨儿童肝脾γδT细胞淋巴瘤(γδHSTCL)的临床表现、细胞形态学(M)、脾活检免疫组织化学(I)、细胞遗传学(C)及TCR基因重排分子生物学(M)特征及其对早期诊断的重要性.方法 分析1例确诊为γδHSTCL患儿的临床及MICM特点,并复习相关文献.结果 骨髓细胞学检查提示约0.365异常细胞浸润;免疫表型分析:异常细胞群约占有核细胞的0.245,主要表达分化群(CD) 2/CD3/CD7/CD19/CD38/eCD3/T细胞受体(TCR)γ/δ.脾脏组织免疫组织化学:CD3(+),CD45RO(+),CD8(+),CD4(-),细胞毒性颗粒相关蛋白(TIA-1)(+),CD20(-),TCRβ(-),CD79α(-),间变性淋巴瘤激酶(ALK)(-),白细胞共同抗原(LCA)(+),CD30(-),末端转移酶(TdT)(-),CD10(-),髓过氧化物酶(MPO)(-),颗粒酶B(Granzyme B)(+);且增殖细胞核抗原(Ki-67)(+)的肿瘤细胞数约为80.00％.脾脏组织流式细胞免疫表型:淋巴细胞占全部有核细胞的72.26％,T淋巴细胞[CD3(+)]约占88.90％,其中约有92.09％的T淋巴细胞Ki-67(+),CD7dim(+),TCRγδ(+),CD2(+),CD8(+),CD34(+),TCRαβ(-),CD5(-),CD4(-),CD56(-),约59.16％细胞为异常成熟T淋巴细胞.骨髓细胞染色体核型:46,XY.骨髓细胞荧光定量聚合酶链反应检测基因重排:TCRβ(+)、兀Rγ(+)和TCRδ(+).患儿入院后先予对症支持治疗及抗感染治疗,确诊后予泼尼松60 mg/m2口服治疗,疗效不佳,死亡.结论 MICM分型对于早期准确诊断和甄别儿童γδHSTCL具有重要价值.

abstractsObjective To investigate the clinic characteristics of hepatosplenic γδT cell lymphoma (γδ HSTCL) and analyze the important role of morphology,immunology,cytogenetics and molecular(MICM) in the early diagnosis of γδHSTCL.Methods The clinical features of MICM of a rare case of childhood γδHSTCL had been analyzed,and the related literatures were reviewed.Results Bone marrow aspiration showed approximately 0.365 abnormal cells infiltrated.The immune phenotype analysis displayed 0.245 of the nuclear cells were abnormal ones,which possessed CD2/CD3/CD7/CD19/CD3s/cCD3 and TCRγδ expression.The immunohistochemical staining of spleen tissues showed clusters of differentiation (CD) 3/CD45 RO/CD8/Cytotoxic-granule associated protein (TIA-1)/leukocyte common antigen (LCA)/Granzyme B positive,CD4/CD20/T cell receptor (TCR) β/CD79 α/CD30/terminal deoxynucleotidy transferase (TdT) /CD10/ myeloperoxidase (MPO)/anaplasticlymphoma kinase (ALK) negative and 80.00％ of tumor cells displayed Ki-67 positive.The detection of spleen tissues by flow cytometry displayed that 72.26％ of the karyocytes were lymphocytes,with 88.90％ T lymphocytes.And 92.09％ of the T lymphocytes showed Ki-67/CD7dim,TCRγδ/CD2/CD8/CD34 positive,and TCRαβ/CD5/CD4/CD56 negative.And about 59.16％ of these karyocytes were abnormally matured T lymphocytes.The cytogenetic karyotype of bone marrow showed 46,XY.The detection of T cell receptor gene rearrangement showed TCRβ,TCRγ and TCRδ positive.The patient had been given prednisone of 60 mg/m2 when the diagnosis of γδHSTCL affirmed.But his parents discontinued chemotherapy for him and died.Conclusions MICM has shown a significant value in the diagnosis of γδHSTCL,a rare lymphoma in childhood.