Takahashi Y, Horibe K, Kiyoi H, Miyashita Y, Fukuda M, Mori H, Nozaki C, Hasegawa S, Kawabe T, Kato K, Kojima S, Matuyama T, Naoe T
Department of Pediatrics, Nagoya University School of Medicine, Japan.
J Pediatr Hematol Oncol. 1998 May-Jun;20(3):190-5. doi: 10.1097/00043426-199805000-00002.
A retrospective study was conducted to investigate the prognostic significance of TEL/AML1 fusion resulting from a cryptic t(12;21) in Japanese patients with childhood B-precursor acute lymphoblastic leukemia (ALL).
Leukemic samples from 144 children with newly diagnosed ALL (104 with CD10-positive B-precursor ALL, 11 with CD10-negative B-precursor ALL, 5 with B-ALL, and 24 with T-ALL) were analyzed by reverse-transcription polymerase chain reaction.
The frequency of patients with TEL/AML1 was 16% (23 of 144) and all patients with TEL/AML1 also had CD10-positive B-precursor ALL. TEL/AML1 was not found in any samples from the patients with T-ALL, B-ALL, or CD10-negative B-precursor ALL. Among patients with CD10-positive B-precursor ALL, age, initial white blood cell count, and immunophenotype did not differ with TEL/AML1 positivity, although the patients were predominantly male (p < 0.01). Clinical outcomes of 94 patients treated with recent protocols were analyzed. Five of the 21 (23.8%) patients with TEL/AML1 relapsed and 4 of these relapsed > 24 months after diagnosis. Although the overall 5-year survival rate was better among patients with TEL/AML1 fusion transcript than among those without it (87.3 +/- 8.7% versus 75.9 +/- 5.8%, respectively), the 5-year disease-free survival (DFS) rates of patients with TEL/AML1 fusion transcript and those without it were similar (64.0 +/- 13.5% versus 69.1 +/- 6.3%, respectively). However, for 57 patients treated with the latest intensive protocol, the 4-year DFS rate was much higher for the patients with TEL/AML1 fusion transcript than for those without it (100.0% v.s. 69.6 +/- 8.4%, respectively, p = 0.1472).
This study confirmed that TEL/AML1 gene fusion is the most common genetic event in pediatric ALL in Japan and is restricted to CD10-positive B-precursor ALL. Moreover, it was associated with an improved survival rate among patients treated with intensive therapy. Therefore, these data suggest that the patients with TEL/AML1 may not necessarily be candidates for less aggressive treatment.
进行一项回顾性研究，以调查隐匿性t(12;21)导致的TEL/AML1融合在日本儿童B前体急性淋巴细胞白血病（ALL）患者中的预后意义。
采用逆转录聚合酶链反应分析144例新诊断ALL患儿（104例CD10阳性B前体ALL、11例CD10阴性B前体ALL、5例B-ALL和24例T-ALL）的白血病样本。
TEL/AML1患者的发生率为16%（144例中的23例），所有TEL/AML1患者均为CD10阳性B前体ALL。T-ALL、B-ALL或CD10阴性B前体ALL患者的任何样本中均未发现TEL/AML1。在CD10阳性B前体ALL患者中，年龄、初始白细胞计数和免疫表型与TEL/AML1阳性与否无关，尽管患者以男性为主（p<0.01）。分析了94例采用近期方案治疗的患者的临床结局。21例TEL/AML1患者中有5例（23.8%）复发，其中4例在诊断后24个月以上复发。虽然TEL/AML1融合转录本阳性患者的总体5年生存率高于无该转录本的患者（分别为87.3±8.7%和75.9±5.8%），但TEL/AML1融合转录本阳性和阴性患者的5年无病生存率（DFS）相似（分别为64.0±13.5%和69.1±6.3%）。然而，对于5
