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Early T-cell precursor leukemia: a subtype of high risk childhood acute lymphoblastic leukemia

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Abstract

Acute lymphoblastic leukemia includes T-cell acute lymphoblastic leukemia (T-ALL) and B-cell acute lymphoblastic leukemia (B-ALL). In children, T-ALL usually has a worse prognosis than B-ALL, although childhood T-ALL prognoses have improved remarkably. The varying outcomes among T-ALL cases suggest that an unrecognized biological heterogeneity may contribute to chemo-resistance. Deep exploration of T-lymphocyte development in recent years has found a subgroup of patients with a phenotype that resembles early T-cell precursor, which confers a much poorer prognosis than any other form of T-ALL. This novel subtype of T-ALL was called early T-cell precursor acute lymphoblastic leukemia (ETP-ALL). Flow cytometry data from T-ALL patients enrolled in Shanghai Children’s Medical Center between July 2002 and October 2010 were assessed according to Dr. Campana’s protocol. Among total 89 T-ALL cases, 74 cases had enough immunophenotype data available to differentiate between ETP (CD1a−, CD8−, CD5dim, at least one marker of stem cell or myeloid lineage) and non-ETP. From these 74 subjects, 12 ETP-ALL cases (16.2%) were identified. The event-free survival (EFS) rate at 66.8 months was 11.1% ± 10.1% for ETP-ALL and 57.6% ± 5.6% for non-ETP-ALL (P = 0.003). The overall survival rates were 13.3% ± 11.0% for ETP-ALL and 64.7% ± 6.3% for non-ETP-ALL (P = 0.002). Our findings demonstrate that early T-cell precursor leukemia is a very high-risk subtype of acute lymphoblastic leukemia with poor prognosis.

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Authors and Affiliations

  1. Department of Hematology/Oncology, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, 200127, China

    Meilin Ma, Xiang Wang, Jingyan Tang, Huiliang Xue, Jing Chen, Ci Pan, Hua Jiang & Shuhong Shen

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  1. Meilin Ma
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  2. Xiang Wang
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  3. Jingyan Tang
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  4. Huiliang Xue
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  5. Jing Chen
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  6. Ci Pan
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  7. Hua Jiang
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  8. Shuhong Shen
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Correspondence to Shuhong Shen.

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Ma, M., Wang, X., Tang, J. et al. Early T-cell precursor leukemia: a subtype of high risk childhood acute lymphoblastic leukemia. Front. Med. 6, 416–420 (2012). https://doi.org/10.1007/s11684-012-0224-4

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  • DOI: https://doi.org/10.1007/s11684-012-0224-4

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