Journal of Clinical Oncology, Vol 15, 2214-2221, Copyright © 1997 by American Society of Clinical Oncology
Clinical features and treatment outcome of childhood T-lineage acute lymphoblastic leukemia according to the apparent maturational stage of T-lineage leukemic blasts: a Children's Cancer Group study
FM Uckun, PS Gaynon, MG Sensel, J Nachman, ME Trigg, PG Steinherz, R Hutchinson, BC Bostrom, HN Sather and GH Reaman
Children's Cancer Group Acute Lymphoblastic Leukemia Biology Reference Laboratory, and Hughes Institute, St. Paul, MN 55113, USA. faith_uckun@mercury.IH.org
PURPOSE: Leukemic cells from T-lineage acute lymphoblastic leukemia (ALL)
patients are thought to originate from T-lymphocyte precursors
corresponding to discrete stages of T-cell ontogeny. Here we sought to
determine the influence of leukemic cell apparent maturational stage on
treatment outcomes in pediatric T-lineage ALL. PATIENTS AND METHODS: From
1983 through 1993, 407 pediatric T-lineage ALL patients were enrolled onto
two sequential series of risk-adjusted treatment protocols of the
Children's Cancer Group. In the current analysis, T- lineage ALL patients
were immunophenotypically classified as follows: CD7+ CD2- CD5-
pro-thymocyte leukemia (pro-TL), CD7+ (CD2 or CD5)+ CD3- immature TL, and
CD7+ CD2+ CD5+ CD3+ mature TL. RESULTS: Similar induction outcomes of
91.4%, 97.1%, and 98.3% were obtained by the pro- , immature, and mature TL
groups, respectively. Four-year event-free survival (EFS) was lower for
pro-TL patients (57.1%; SD = 8.4%,) compared with immature and mature TL
patients (68.5%; SD = 3.5%; and 77.1%; SD = 4.0%, respectively) with an
overall significance of .05 (log-rank test) or .04 (log-rank trend test).
Relative hazards rates (RHR) were 2.11 and 1.22 for pro-TL and immature TL
versus mature TL, respectively. Highly significant differences were found
for overall survival (P = .005, log-rank test; P = .009, log-rank trend
test). Multivariate analysis confirmed that the prognostic influence of
ontogeny grouping was independent of that of other prognostic factors.
CONCLUSION: Leukemic cells of the pro-TL maturation stage identify a small
subgroup of T-lineage ALL patients who have a significantly worse EFS
outcome than patients whose cells are of a more mature stage of
development.
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