CHRONIC MYELOGENOUS LEUKEMIA IN CHILDREN

Abstract

We have reviewed the 40 cases of adult-type chronic myelogenous leukemia (ACML) and 21 cases of juvenile chronic myelogenous leukemia (JCML) seen at St. Jude Children's Research Hospital since 1964. The ACML patients ranged in age from 9 mo to 20-4/12 yr (median 11-1/12 yr) and included 23 boys and 17 girls. The JCML patients ranged from 6 mo to 11-8/12 yr (median 2 yr) and included 14 boys and 7 girls. Cytogenetic studies from 32 of the 36 ACML patients demonstrated the Philadelphia chromosome, whereas studies from only 1 of the 15 JCML patients had a structural chromosome abnormality. The range and median of other presenting characteristics are summarized below:The survival of the ACML patients ranged from 5 to 63 mo from diagnosis (median 25) and for the JCML patients from 2 to 28 mo (median 9). Eight ACML patients remain alive in chronic phase, 4 died in chronic phase, 5 had disease acceleration without clear documentation of blast crisis, 23 entered blast crisis 4 to 63 mo after diagnosis (6 lymphoblastic, 17 nonlymphoblastic). Five of the 6 patients entering lymphoblastic crisis achieved remission after 4 weeks of a vincristine-prednisone containing regimen. All 21 JCML patients died of disease despite modern therapy. Our studies suggest that better characterization of blast crisis cells of ACML patients may permit more specific and effective therapy. New therapeutic approaches are needed for both ACML and JCML.