Prognostic factors in childhood acute lymphoblastic leukemia in Japan. Japan Association of Childhood Leukemia Study.

A retrospective analysis of children with acute lymphoblastic leukemia (ALL) was performed to evaluate the current status of diagnosis and treatment of 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 Association of Childhood Leukemia Study. It was found that T-cell ALL was significantly less frequent in Tokai and Hokkaido than in Kansai and Chu-Shikoku. The overall induction rate was 92.4%. The estimated 7-year overall survival rate and event-free survival (EFS) rate were 76.0% +/- 1.9% and 61.4% +/- 2.1%, respectively. EFS rates were significantly different among the geographic areas. In female patients with B-cell precursor (B-pre) ALL and white blood cell counts at diagnosis (WBCsdiag) below 50.0 x 10(9)/L, favorable outcomes 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. The EFS rate for 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 higher the WBCdiag, the worse the EFS rate. Multivariate analysis showed that the following factors were significantly unfavorable for EFS: the Philadelphia chromosome, an translocations associated with chromosome 11q23, an acute 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 outcome in childhood ALL, a prospective large-scale study should be undertaken in Japan.