A retrospective analysis of children with acute lymphoblastic leukemia (ALL
) was performed to evaluate the current status of diagnosis and treatment o
f ALL in Japanese children. Clinical records of 670 children with ALL were
collected and analyzed; these children had been diagnosed between 1991 and
1995 at the 53 institutions in 4 areas participating in the Japan Associati
on of Childhood Leukemia Study. It was found that T-cell ALL was significan
tly less frequent in Tokai and Hokkaido than in Kansai and Chu-Shikoku. The
overall induction rate was 92.31%.The estimated 7-year overall survival ra
te and event-free survival (EFS) rate were 76.0% +/- 1.9% and 61.4% +/- 2.1
%, respectively. EFS rates were significantly different among the geographi
c areas. In female patients with B-cell precursor (B-pre) ALL and white blo
od cell counts at diagnosis (WBCsdiag) below 50.0 x 10(9)/L, favorable outc
omes were significant. Favorable outcomes were not significant in B-pre ALL
patients with a WBCdiag above 50.0 x 10(9)/L or in T-cell ALL patients. Th
e EFS rate fur infants was significantly worse than that for patients over
1 year of age. In B-pre ALL, but not in T-cell ALL, it was found that the h
igher the WBCdiag, the worse the EFS rate. Multivariate analysis showed tha
t the following factors were significantly unfavorable for EFS: the Philade
lphia chromosome, an translocations associated with chromosome 11q23, an ac
ute unclassified leukemia, mixed-lineage leukemia, a WBCdiag above 100.0 x
10(9)/L, and male gender. Hyperdiploidy (>50 chromosomes) was significantly
favorable for EFS. For further tailoring of treatment and to improve the o
utcome in childhood ALL, a prospective large-scale study should be undertak
en in Japan. Int J Hematol 2000,72:61-68. (C) 2000 The Japanese Society of
Hematology.