儿童急性淋巴细胞白血病TEL-AML1阳性与阴性临床特征的比较研究
[Comparative study on clinical features between TEL-AML1 positive and negative childhood acute lymphoblastic leukemia].
作者信息
Guo Xia, Li Qiang, Zhu Yi-ping, Zhou Chen-yan, Gao Ju, Li Xi-hong, Pan Ling-li, Li Feng-yi, Tian Xin, Liu Hai-ting
机构信息
Department of Pediatrics, West China Second Hospital, Sichuan University, Chengdu, Sichuan, 610041 PR China.
出版信息
Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2007 Oct;24(5):560-3.
OBJECTIVE
To determine the incidence of TEL-AML1 fusion gene in childhood acute lymphoblastic leukemia (ALL) and to compare the clinical features between TEL-AML1 positive and negative patients.
METHODS
Samples of bone marrow or peripheral blood were collected from 95 newly diagnosed ALL children and the TEL-AML1 fusion gene was detected using nested reverse transcription-polymerase chain reaction (RT-PCR). The ALL patients were stratified into TEL-AML1 positive and negative groups and the clinical features were compared.
RESULTS
Among 95 patients, 20 (21.05%) were TEL-AML1 positive. The median age of TEL-AML1 positive patients was 5.9 years old and M/F ratio was 1.22:1. There were significant differences between TEL-AML1 positive and negative patients in hepatomegaly (2.75 cm vs. 4 cm below costal arch, P=0.006), splenomegaly (0 cm vs. 3 cm below costal arch, P < 0.001), initial white blood cell count (median 7.40 x 10(9)/L vs.18.70 x 10(9)/L, P=0.011), initial peripheral blood blast (median 2.45 x 10(9)/L vs.11.66 x 10(9)/L, P=0.013), hemoglobin level [(61.45 +/- 13.46) g/L vs. (75.89 +/- 23.11) g/L, P=0.003] and serum lactate dehydrogenase [(621.47 +/- 335.85) U/L vs.(1566.64 +/- 1720.45) U/L, P=0.020], while no differences were found between two groups in age, gender ratio, initial platelet count, percentage of blast in bone marrow, immunophenotypes and the expression of myeloid antigen CD13, CD33 and CD34. The prednisone sensitivity test showed that all 12 TEL-AML1 positive patients were good responders, while there were 11 prednisone poor responders among 40 negative patients (27.50%, P < 0.05). Bone marrow examination on day 15 showed no difference in the rate of complete remission between TEL-AML1 positive and negative patients.
CONCLUSION
The incidence of TEL-AML1 fusion gene in cases of ALL is 21.05%. The load of leukemia cells in TEL-AML1 positive patients is significantly smaller than its counterparts, and the blast cells in TEL-AML1 positive patients are more sensitive to prednisone, indicating childhood ALL with TEL-AML1 fusion gene has a favorable prognosis.
目的
确定儿童急性淋巴细胞白血病（ALL）中TEL-AML1融合基因的发生率，并比较TEL-AML1阳性和阴性患者的临床特征。
方法
收集95例新诊断的ALL儿童的骨髓或外周血样本，采用巢式逆转录聚合酶链反应（RT-PCR）检测TEL-AML1融合基因。将ALL患者分为TEL-AML1阳性和阴性组，比较其临床特征。
结果
95例患者中，20例（21.05%）TEL-AML1阳性。TEL-AML1阳性患者的中位年龄为5.9岁，男女比例为1.22:1。TEL-AML1阳性和阴性患者在肝肿大（肋弓下2.75 cm对4 cm，P=0.006）、脾肿大（肋弓下0 cm对3 cm，P<0.001）、初始白细胞计数（中位7.40×10⁹/L对18.70×10⁹/L，P=0.011）、初始外周血原始细胞（中位2.45×10⁹/L对11.66×10⁹/L，P=0.013）、血红蛋白水平[（61.45±13.46）g/L对（75.89±23.11）g/L，P=0.003]和血清乳酸脱氢酶[（621.47±335.85）U/L对（1566.64±1720.45）U/L，P=0.020]方面存在显著差异，而两组在年龄、性别比例、初始血小板计数、骨髓原始细胞百分比、免疫表型以及髓系抗原CD13、CD33和CD34的表达方面无差异。泼尼松敏感性试验显示，12例TEL-AML1阳性患者均为泼尼松良好反应者，而40例阴性患者中有11例泼尼松反应不佳者（27.50%，P<0.05）。第15天的骨髓检查显示，TEL-AML1阳性和阴性患者的完全缓解率无差异。
结论
ALL病例中TEL-AML1融合基因的发生率为21.05%。TEL-AML1阳性患者的白血病细胞负荷明显低于阴性患者，且TEL-AML1阳性患者的原始细胞对泼尼松更敏感，提示伴有TEL-AML1融合基因的儿童ALL预后良好。
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