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目的:探讨胞浆μ重链(Cμ)检测在急性非成熟B淋巴细胞白血病治疗及预后中的指导意义,为寻找可能的合理的治疗方案提供依据。方法:非成熟B淋巴细胞白血病患儿161例,按ALL-XH-99方案化疗。并在治疗前获得患者年龄、性别、外周血白细胞数、免疫分型、P170、融合基因以及强的松治疗第8天外周血幼稚细胞绝对数、诱导缓解治疗第18天骨髓象以及诱导缓解治疗结束时骨髓MRD(微小残留病)水平和危险度分级等,并动态观测治疗疗效。结果:Kaplan-Meier分析显示,Cμ阳性患者5年EFS为(56.7±0.88)%,而Cμ阴性患者(Pro-B和Common B)则达到(74.1±0.05)%。两者差异显著(P<0.01);χ2分析显示,两组之间的预后差异与患者的生物学特征如性别、年龄、初诊时外周血白细胞(WBC)计数、P170水平、有无髓系标记(My)、融合基因(是否具有BCR/ABL或MLL/AF4)、强的松窗口实验以及治疗第19天骨髓象等无相关性;COX回归风险分析显示,Cμ阳性、治疗前外周血白细胞数WBC≥50×109/L、MRD阳性以及融合基因检测阳性等具有独立预后意义(P<0.05)。结论:Cμ阳性在儿童非成熟B淋巴细胞性白血病化疗中具有重要的不良预后意义,而且这种不良预后与CD10阴性MLL基因重排之间并无相关性。
Objective: To investigate the significance of detection of cytoplasmic μ heavy chain (Cμ) in the treatment and prognosis of acute non-mature B lymphoblastic leukemia and to provide a basis for finding a possible reasonable treatment plan. Methods: 161 children with immature B-cell leukemia were treated with ALL-XH-99 chemotherapy. The patient’s age, sex, peripheral blood leukocyte count, immunophenotype, P170, fusion gene and the absolute number of naive cells in peripheral blood of prednisone on the 8th day were obtained before treatment, and the bone marrow was induced on the 18th day after induction therapy and induced remission therapy At the end of bone marrow MRD (minimal residual disease) levels and risk grading, and dynamic observation of treatment efficacy. Results: Kaplan-Meier analysis showed that the 5-year EFS was 56.7 ± 0.88% in Cμ-positive patients and 74.1 ± 0.05% in Cμ-negative patients (Pro-B and Common B). There was a significant difference between the two groups (P <0.01). Chi-square analysis showed that the difference between the two groups was not related to the patient’s biological characteristics such as gender, age, WBC count, P170 level, (My), fusion gene (whether with BCR / ABL or MLL / AF4), prednisone window test and treatment of bone marrow on the 19th day and so no correlation; COX regression risk analysis showed that Cμ positive, pre-treatment peripheral blood leukocytes WBC≥50 × 109 / L, positive MRD and positive fusion gene have the independent prognostic significance (P <0.05). CONCLUSIONS: Cμ-positivity has an important negative prognostic significance in the chemotherapy of childhood non-mature B-lymphoblastic leukemia, and this poor prognosis is not associated with CD10-negative MLL gene rearrangement.