Pediatric acute lymphoblastic leukemia with t(1;19)/TCF3-PBX1 in Taiwan
PEDIATRIC BLOOD & CANCER
Authors: Yen, Hsiu-Ju; Chen, Shih-Hsiang; Chang, Tsung-Yen; Yang, Chao-Ping; Lin, Dong-Tsamn; Hung, Iou-Jih; Lin, Kai-Hsin; Chen, Jiann-Shiuh; Hsiao, Chih-Cheng; Chang, Tai-Tsung; Chang, Te-Kao; Peng, Ching-Tien; Lin, Ming-Tsan; Jaing, Tang-Her; Liu, Hsi-Che; Jou, Shiann-Tarng; Lu, Meng-Yao; Cheng, Chao-Neng; Sheen, Jiunn-Ming; Chiou, Shyh-Shin; Hung, Giun-Yi; Wu, Kang-Hsi; Yeh, Ting-Chi; Wang, Shih-Chung; Chen, Rong-Long; Chang, Hsiu-Hao; Yang, Yung-Li; Chen, Shu-Huey; Cheng, Shin-Nan; Chang, Yu-Hsiang; Chen, Bow-Wen; Hsieh, Yuh-Lin; Huang, Fang-Liang; Ho, Wan-Ling; Wang, Jinn-Li; Chang, Chia-Yau; Chao, Yu-Hua; Lin, Pei-Chin; Chen, Yu-Chieh; Liao, Yu-Mei; Lin, Tung-Huei; Shih, Lee-Yung; Liang, Der-Cherng
Abstract
BackgroundIn childhood acute lymphoblastic leukemia (ALL), t(1;19)(q23;p13.3) with TCF3-PBX1 fusion is one of the most frequent translocations. Historically, it has been associated with poor prognosis. Intensive treatment, however, has improved its outcome. We determined the outcome of children with this genotype treated with contemporary intensive chemotherapy in Taiwan. ProcedureIn Taiwan Pediatric Oncology Group 2002 ALL studies, genotypes were determined by cytogenetic analysis and/or reverse transcriptase polymerase chain reaction assay. Based on presenting features, immunophenotype and genotype, patients were assigned to one of the three risk groups: standard risk (SR), high risk (HR), or very high risk (VHR). The patients with t(1;19)/TCF3-PBX1 were treated in the HR arm receiving more intensive chemotherapy. The outcomes of patients with t(1;19)/TCF3-PBX1 were compared to that of patients with other subtypes of B-precursor ALL (B-ALL). ResultsOf the 1,129 patients with B-ALL, 64 (5.7%) had t(1;19)/TCF3-PBX1; 51 of whom were treated in the HR arm, but 11 were treated in the VHR and 2 in the SR arm because of physician's preference. As a group, 64 patients with t(1;19)/TCF3-PBX1 had similar 5-year event-free survival (83.3 4.8%) as those with TEL-AML1 (85.2 +/- 3.4%, P = 0.984) or those with hyperdiploidy >50 (84.0 +/- 3.1%, P = 0.748). The cumulative risk of any (isolated plus combined) central nervous system relapse among patients with t(1;19)/TCF3-PBX1 (8.7 +/- 3.8%) tended to be higher than that of patients with TEL-AML1 (5.8 +/- 2.3%, P = 0.749) or those with hyperdiploidy (4.1 +/- 1.8%, P = 0.135), albeit the differences did not reach statistical significance. ConclusionsWith contemporary intensive chemotherapy, children with t(1;19)/TCF3-PBX1 fared as well as those with favorable genotypes (TEL-AML1 or hyperdiploidy).
Clinical Features and Prognostic Significance of TCF3-PBX1 Fusion Gene in Chinese Children with Acute Lymphoblastic Leukemia by Using a Modified ALL-BFM-95 Protocol
PEDIATRIC HEMATOLOGY AND ONCOLOGY
Authors: Pang, Li; Liang, Ying; Pan, Jian; Wang, Jian-Rong; Chai, Yi-Huan; Zhao, Wen-Li
Abstract
For children with precursor B (pre-B) acute lymphoblastic leukemia (ALL) with TCF3-PBX1 fusion gene, their prognosis has been a controversial topic. From January 2008 to December 2012 in our hospital, 450 patients were diagnosed as ALL. Clinical characteristics of 20 patients with TCF3-PBX1 fusion gene were analyzed retrospectively, which were classified to the intermediate-risk (IR) group according to Chinese Children Leukemia Group-2008 (CCLG-2008) risk-stratification criteria and protocol based on the backbone of BFM 95 trails. Eighty five cases without TCF3-PBX1 in the same IR group were regarded as the comparison group. There were no differences in age, gender, initial white blood cell (WBC) count, status of central nerves system (CNS) at diagnosis and complete remission (CR) rates of bone marrow (BM) between the two groups (P > .05). The 5-year probability of event-free survival (EFS) rates were 84.4 +/- 15.6% and 73.5 +/- 15.6% in the TCF3-PBX1 group and the comparison group (P =.35), respectively. The 5-year probability of overall survival (OS) rates were 86.0 +/- 17.6% and 81.8 +/- 17.6% (P = .46), respectively. Relapse rates were 10.5% and 12.9% (P = 1.00), respectively. There were not cases with CNS relapse in the TCF3-PBX1 group. When intensive chemotherapy was used, the TCF3-PBX1 was associated with a favorable outcome in childhood pre-B ALL.