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Hyperdiploid (47-50) acute lymphoblastic leukemia in children
SC Raimondi, PK Roberson, CH Pui, FG Behm and GK Rivera
Departments of Pathology and Laboratory Medicine, St Jude Children's
Research Hospital, Memphis, TN 38105.
Among ploidy groups in childhood acute lymphoblastic leukemia (ALL),
hyperdiploidy 47 to 50 is perhaps the least well known. From December 1979
to December 1990, we successfully studied banded karyotypes in 598 cases of
newly diagnosed ALL, of which 86 (14.4%) had modal chromosome numbers of 47
to 50. In this group, the most frequently acquired numerical abnormalities
were +21 (n = 34), +X (18), +8 (8), and +10 (7). The chromosomal regions
most often affected by structural abnormalities were 1q (n = 13), 6q (12),
12p (18), and 19p (9). Analysis of event-free survival (EFS) for Studies X
and XI among patients with hyperdiploid (47 to 50) ALL showed no
significant differences in outcome according to the presence (n = 36) or
absence (n = 35) of chromosomal translocations (P = .81) or the gain of
specific chromosomes (P = .40). Patients with hyperdiploid (47 to 50) ALL
treated in a contemporary program of multiagent chemotherapy had a
significantly better outcome than did those in an earlier study using less
intensive therapy (4-year EFS = 75% [95% confidence interval, 55% to 86%] v
41% [22% to 59%]; P = .006 by the logrank test). Our findings indicate that
the adverse prognosis previously attributed to hyperdiploidy 47 to 50
improves significantly with more effective chemotherapy.
Volume 79,
Issue 12,
pp. 3245-3252,
06/15/1992
Copyright © 1992 by The American Society of Hematology
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