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Next Article 
Biology and clinical significance of cytogenetic abnormalities in childhood
acute lymphoblastic leukemia
CH Pui, WM Crist and AT Look
Department of Hematology-Oncology, St Jude Children's Research Hospital,
Memphis, TN 38101.
Virtually all cases of childhood ALL have chromosomal abnormalities and
half contain translocations, which are nearly equally divided between
random and nonrandom rearrangements. Nonrandom chromosomal abnormalities
have been correlated with leukemic cell lineage, the degree of cell
differentiation, and the specific gene involved at the molecular level.
Many cytogenetic findings have prognostic significance; however, the
adverse influence of certain changes, including most chromosomal
translocations, may in fact be offset by the greater cytoreductive effects
of intensified therapy. Table 4 summarizes the relation of selected
karyotypic findings to treatment outcome in patients treated on
contemporary protocols. Among all of the chromosomal abnormalities
identified in childhood ALL, hyperdiploidy greater than 50 has been
associated with the most favorable prognosis. At the opposite end of the
spectrum, the treatment outcome for patients with classical Ph+ or
hypodiploid ALL is very poor even in programs of intensive chemotherapy;
alternative treatment such as bone marrow transplantation should be
considered for such patients. Cases with the t(4;11)(q21;q23) also have a
very poor clinical outcome, but the adverse prognosis may be limited to the
infant or adolescent age groups. The prognostic significance of other
nonrandom translocations, such as t(1;19)(q23;p13) and several other
abnormalities, needs to be further assessed in larger numbers of patients.
Finally, as more is learned about the molecular pathology underlying these
rearrangements, it may be possible to develop new therapeutic agents that
are specifically targeted to interfere with the aberrant gene products
expressed by human leukemic cells.
Volume 76,
Issue 8,
pp. 1449-1463,
10/15/1990
Copyright © 1990 by The American Society of Hematology
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