Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus: A pediatric oncology group study

Robert O. Bash, William M. Crist, Jonathan J. Shuster, Michael P. Link, Michael Amylon, Jeanette Pullen, Andrew J. Carroll, George R. Buchanan, R. Graham Smith, Richard Baer

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Abstract

Alteration of the TAL1 locus is the most common nonrandom genetic defect in childhood T-cell acute lymphoblastic leukemia (T-ALL). To determine if rearrangements of the TAL1 proto-oncogene confer a distinct leukemic phenotype, we studied leukemic peripheral blood or bone marrow samples from 182 children with newly diagnosed T-ALL enrolled on Pediatric Oncology Group treatment protocols. Forty-eight (26%) of the samples had a local rearrangement of the TAL1 locus. Demographic and clinical features were compared for patient subgroups with and without TAL1 rearrangements. The only clinical correlates that were significantly associated with TAL1 gene rearrangements were higher white blood cell count (P = .017) and higher hemoglobin (P = .007) at diagnosis. Immunophenotypically, samples with altered TAL1 were more likely to be CD2+ (P = .001) and lack CD10 (cALLa) expression (P = .007) than those without the rearrangement. There was a trend toward improved event-free survival (EFS) in patients with TAL1 rearrangements (4-year EFS was 44% ± 7% for patients without the rearrangements v 59% ± 11% for those with rearrangements), but the difference was not significant (P = .34). The role of TAL1 in leukemogenesis has yet to be clearly defined, and the prognostic significance of TAL1 gene rearrangements in T-ALL deserves further study.

Original languageEnglish (US)
Pages (from-to)2110-2117
Number of pages8
JournalBlood
Volume81
Issue number8
StatePublished - Apr 15 1993

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Pediatrics
Oncology
T-cells
Precursor Cell Lymphoblastic Leukemia-Lymphoma
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
T-Lymphocytes
Disease-Free Survival
Blood
Genes
T-Lymphocyte Gene Rearrangement
Proto-Oncogenes
Gene Rearrangement
Clinical Protocols
Leukocyte Count
Bone
Bone Marrow
Cells
Demography
Phenotype
Defects

ASJC Scopus subject areas

  • Hematology

Cite this

Bash, R. O., Crist, W. M., Shuster, J. J., Link, M. P., Amylon, M., Pullen, J., ... Baer, R. (1993). Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus: A pediatric oncology group study. Blood, 81(8), 2110-2117.

Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus : A pediatric oncology group study. / Bash, Robert O.; Crist, William M.; Shuster, Jonathan J.; Link, Michael P.; Amylon, Michael; Pullen, Jeanette; Carroll, Andrew J.; Buchanan, George R.; Smith, R. Graham; Baer, Richard.

In: Blood, Vol. 81, No. 8, 15.04.1993, p. 2110-2117.

Research output: Contribution to journalArticle

Bash, RO, Crist, WM, Shuster, JJ, Link, MP, Amylon, M, Pullen, J, Carroll, AJ, Buchanan, GR, Smith, RG & Baer, R 1993, 'Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus: A pediatric oncology group study', Blood, vol. 81, no. 8, pp. 2110-2117.
Bash, Robert O. ; Crist, William M. ; Shuster, Jonathan J. ; Link, Michael P. ; Amylon, Michael ; Pullen, Jeanette ; Carroll, Andrew J. ; Buchanan, George R. ; Smith, R. Graham ; Baer, Richard. / Clinical features and outcome of T-cell acute lymphoblastic leukemia in childhood with respect to alterations at the TAL1 locus : A pediatric oncology group study. In: Blood. 1993 ; Vol. 81, No. 8. pp. 2110-2117.
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abstract = "Alteration of the TAL1 locus is the most common nonrandom genetic defect in childhood T-cell acute lymphoblastic leukemia (T-ALL). To determine if rearrangements of the TAL1 proto-oncogene confer a distinct leukemic phenotype, we studied leukemic peripheral blood or bone marrow samples from 182 children with newly diagnosed T-ALL enrolled on Pediatric Oncology Group treatment protocols. Forty-eight (26{\%}) of the samples had a local rearrangement of the TAL1 locus. Demographic and clinical features were compared for patient subgroups with and without TAL1 rearrangements. The only clinical correlates that were significantly associated with TAL1 gene rearrangements were higher white blood cell count (P = .017) and higher hemoglobin (P = .007) at diagnosis. Immunophenotypically, samples with altered TAL1 were more likely to be CD2+ (P = .001) and lack CD10 (cALLa) expression (P = .007) than those without the rearrangement. There was a trend toward improved event-free survival (EFS) in patients with TAL1 rearrangements (4-year EFS was 44{\%} ± 7{\%} for patients without the rearrangements v 59{\%} ± 11{\%} for those with rearrangements), but the difference was not significant (P = .34). The role of TAL1 in leukemogenesis has yet to be clearly defined, and the prognostic significance of TAL1 gene rearrangements in T-ALL deserves further study.",
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