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目的:提高对前体T淋巴母细胞白血病/淋巴瘤(T-LBL/ALL)的认识。方法:报道1例T-LBL/ALL,并对国内外相关文献进行复习。结果:患者持续咳嗽、低热、颜面部水肿,前纵膈病理提示前纵膈T淋巴母细胞淋巴瘤。免疫组织化学:肿瘤细胞CD3、CD7、TDT、CD34、CD99均(+),CD43散在(+),CD20、PAX5、CD2、PCK、EMA、CK8/18均(-),KI-67LI 50%左右,骨髓中原始、幼稚淋巴细胞占92.5%,骨髓流式细胞考虑为恶性T系淋巴细胞伴CD117、CD56表达,T淋巴细胞淋巴瘤/白血病可能性大,骨髓融合基因阴性,检测到TCRγ呈单克隆性重排的抗原受体基因条带,染色体为高度复杂混合异常克隆,符合T-LBL/ALL的诊断标准。对化疗反应差,自动要求出院。结论:T-LBL/ALL比较少见,仅凭临床表现很难确诊,遇到疑似病例,应做组织病理活检、细胞免疫表型分析、受体基因重排、细胞遗传学以明确诊断。T-LBL/ALL采用Hyper-CVAD方案及T-ALL方案,诱导缓解率比较高,并且进行常规的中枢神经系统预防至关重要。
OBJECTIVE: To improve the understanding of precursor T-cell lymphoblastic leukemia / lymphoma (T-LBL / ALL). Methods: One case of T-LBL / ALL was reported, and the relevant literature at home and abroad was reviewed. Results: The patient continued to cough, fever, facial edema, the first mediastinal pathology prompted mediastinal T lymphoblastic lymphoma. Immunohistochemistry: CD3, CD7, TDT, CD34, CD99 (+), CD43 scattered (+), CD20, PAX5, CD2, PCK, EMA, CK8 / , Bone marrow in naive, naive lymphocytes accounted for 92.5%, bone marrow flow cytometry considered as malignant T lymphocytes with CD117, CD56 expression, T lymphocytic lymphoma / leukemia, bone marrow fusion gene was negative, TCRγ was detected as a single Clonal rearrangement of the antigen receptor gene bands, chromosomal abnormalities are highly complex mixed clone, in line with the T-LBL / ALL diagnostic criteria. Poor response to chemotherapy, automatic discharge. Conclusion: T-LBL / ALL is relatively rare. Clinical manifestations are difficult to diagnose. In case of suspected cases, histopathological biopsy, cellular immunophenotyping, receptor gene rearrangement and cytogenetics should be used to confirm the diagnosis. T-LBL / ALL with Hyper-CVAD program and T-ALL program, induced remission rate is relatively high, and for the prevention of the central nervous system is essential.