Pediatric acute lymphoblastic leukemia with t(1;19)/TCF3-PBX1 in Taiwan

作者：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

来源：Pediatric Blood and Cancer, 2017, 64(10): e26557.

DOI：10.1002/pbc.26557

• clinical studies
• CNS leukemia
• pediatric B-precursor ALL
• relapse
• Taiwanese
• TCF3-PBX1

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).

• 出版日期2017-10
• 单位长春大学; 中国医科大学