急性白血病患儿Notch1蛋白表达及其临床意义研究

摘要：目的　探讨儿童急性白血病（AL）骨髓细胞中Notch1蛋白表达及其与免疫分型、治疗效果的关系。方法　收集2007年1月至2009年1月华中科技大学同济医学院附属同济医院初诊49例AL患儿和20例非恶性血液病患儿骨髓涂片，采用免疫组织化学链霉素亲和素-生物素-过氧化酶复合物（SABC）方法检测骨髓细胞中Notch1蛋白表达情况；流式细胞仪确定白血病细胞免疫分型；标准化疗方案用于AL患儿的治疗。结果　Notch1在T淋巴细胞性白血病（T-ALL）患儿阳性表达率为77.8%，显著高于B淋巴细胞白血病（B-ALL）患儿（31.0%，P < 0.05）及对照组骨髓组织（15.0%，P < 0.05）。急性非淋巴细胞白血病（ANLL）Notch1蛋白阳性表达率为63.6%，显著高于对照组（15.0%，P < 0.05）。在T-ALL 和ANLL中，Notch1与治疗效果无明显相关性；但在随访半年以上的B-ALL中，治疗效果好组（完全缓解无复发）Notch1阳性表达率为21.4%，效果差组（死亡或复发）Notch1阳性表达率为80.0%，两者比较差异有统计学意义（P < 0.05）。结论　Notch1在儿童AL中的异常表达与免疫分型关系密切。Notch1蛋白在B-ALL中虽然表达率不高，但可能是预后不良的因素。

Significance of Notch1 protein expression in pediatric acute leukemia.

Abstract：Objective　To investigate the expression of Notch1 protein in the children with acute leukemia （AL） and to explore the relationship of Notch1 expression with immunophenotype and therapeutic effect. Methods　The Notch1 protein of bone marrow smears was examined by immunohistochemical assay in 49 newly diagnosed children with acute leukemia and 20 control cases without malignant hematologic disease. Immunophenotype of leukemic cells was performed by flow cytometry. The standard chemotherapy regimens were taken to treat the patients. Results　The expression rate of Notch1 protein in T-cell acute lymphoblastic leukemia （T-ALL） （77.8%） was much higher than that of B-cell acute lymphoblastic leukemia （B-ALL） （31.0%， P < 0.05） as well as the control group （（15.0%， P < 0.05）. Notch1 protein expression in acute non-lymphoblastic leukemia （ANLL） （63.6%） was significantly higher than that in the control group （15.0%， P < 0.05）. Additionally， in T-ALL and ANLL groups， there was no correlation between the expression of Notch1 protein and treatment effect. However， in B-ALL patients with more than 6 months of follow-up， Notch1 expression rates in the favorable group（complete remission without relapse） and the unfavorable group （death or relapse） were 21.4%and 80.0% respectively （P < 0.05）. Conclusion　Notch1 expression in childhood leukemia is immunophenotype-specific. Though the expression of Notch1 protein in B-ALL is not high， it may be a poor prognosis factor.