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Clinical features and outcome in childhood T-cell leukemia-lymphoma
according to stage of thymocyte differentiation: a Pediatric Oncology Group
Study
WM Crist, JJ Shuster, J Falletta, DJ Pullen, CW Berard, TJ Vietti, CS Alvarado, MA Roper, E Prasthofer and CE Grossi
Department of Hematology-Oncology, St Jude Children's Research Hospital,
Memphis, TN 38101.
The immunophenotypes of lymphoblasts from children with newly diagnosed
T-cell acute lymphoid leukemia (T-ALL, n = 101) or T-cell non-Hodgkin
lymphoma (T-NHL, n = 31) were analyzed to correlate stage of thymocyte
differentiation with clinical features and outcome. The 67 boys and 34
girls with T-ALL were 1 month to 18 years old (median, 8 years) with
leukocyte counts ranging from 2 to 810 x 10(9)/L (median, 55 x 10(9)/L).
Eighteen of these patients were black, and 70 had a mediastinal mass.
Twenty-six boys and five girls with a median age of 9 years (range, 1 to 20
years) had T-NHL. Seven of these patients were black, and 24 had a
mediastinal mass. The distributions of thymocyte developmental stages
(early [CD7+], intermediate [CD1+ and/or CD4+ and/or CD8+], and mature
[CD3+]) in cases of T-ALL and T-NHL were significantly different: 34%, 43%,
and 23% v 6%, 62%, and 32% (P = .02). A comparison of the patients'
clinical features according to the maturational stage of thymocytes failed
to disclose significant differences in the majority of characteristics
studied. However, patients with mature-stage T-NHL, with or without the
addition of subjects with mature-stage T-ALL, were less likely to have a
mediastinal mass (P = .02 for both comparisons). Those with
intermediate-stage T-cell malignancy (T-ALL and T-NHL combined) were the
subgroup most likely to have a mediastinal mass (P = .01). Response to
remission induction therapy was significantly worse in the T-ALL subgroup
with an early-stage phenotype: a failure rate of 21% v 0% and 6% for the
two more differentiated phenotypic subgroups (P = .007). Event-free
survival was not affected by thymocyte maturational stage in cases of
either T-ALL or T-NHL. Despite evidence of clinical heterogeneity among the
maturational stages of T-cell malignancies in children, these developmental
subdivisions do not appear to be critical determinants of outcome once
remission is achieved. We conclude that such phenotypes need not be
included in the stratification plans for clinical trials using common
induction treatment.
Volume 72,
Issue 6,
pp. 1891-1897,
12/01/1988
Copyright © 1988 by The American Society of Hematology
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