Absence of biallelic TCRγ deletion predicts induction failure and poorer outcomes in childhood T-cell acute lymphoblastic leukemia

Yung Li Yang, Chih Cheng Hsiao, Hsuan Yu Chen, Kai Hsin Lin, Shiann Tarng Jou, Jiann Shiuh Chen, Te Kau Chang, Jiunn Ming Sheen, Sung Liang Yu, Meng Yao Lu, Chao Neng Cheng, Kang Hsi Wu, Shih Chung Wang, Jiaan Der Wang, Hsiu Hao Chang, Shu Rung Lin, Shu Wha Lin, Dong Tsamn Lin

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9 Citations (Scopus)

Abstract

Background: The absence of biallelic TCRγ deletion (ABD) is a characteristic of early thymocyte precursors before V(D)J recombination. The ABD was reported to predict early treatment failure in T-cell acute lymphoblastic leukemia (ALL). This study aimed to investigate its prognostic value in Taiwanese patients with T-cell ALL. Procedure: Forty-five children with T-cell ALL were enrolled from six medical centers in Taiwan. Quantitative DNA polymerase chain reaction (Q-PCR) was performed to check the status of TCRγ deletion. The threshold for homozygous deletions by Q-PCR was defined as a fold-change <0.35. Results: ABD was found in 20 patients [20:45] who had higher incidences of induction failure than those without ABD (P=0.03; hazard ratio [HR]=8.13; 95% confidence interval [95% CI]=1.23-53.77) after multivariate regression analysis. Patents with ABD also had inferior EFS and OS (P=0.071 and 0.0196, respectively). Multivariate Cox analysis indicated that the association between ABD and overall survival was independent of age and leukocyte count on presentation (P=0.036; HR=4.25; 95% CI=1.10-16.42). Conclusions: The absence of TCRγ deletion is a predictor of a poor response to induction chemotherapy for pediatric patients with T-cell ALL in Taiwan. Providing patients with T-cell ALL and ABD with alternative regimens may be worthwhile to test in future clinical trials.

Original languageEnglish
Pages (from-to)846-851
Number of pages6
JournalPediatric Blood and Cancer
Volume58
Issue number6
DOIs
Publication statusPublished - 2012 Jun

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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