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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
RO Bash, WM Crist, JJ Shuster, MP Link, M Amylon, J Pullen, AJ Carroll, GR Buchanan, RG Smith and R Baer
Department of Pediatrics, University of Texas Southwestern Medical Center,
Dallas 75235-9048.
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.
Volume 81,
Issue 8,
pp. 2110-2117,
04/15/1993
Copyright © 1993 by The American Society of Hematology
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