Philadelphia chromosome positive childhood acute lymphoblastic leukemia.
In a 3-yr period, the Philadelphia chromosome (Ph1) was found in 4 of 43 children with acute lymphoblastic leukemia (ALL) in whom chromosomes were studied at diagnosis. The clinical, morphological, cytochemical, and immunologic findings in the Ph1-positive (PH1+) CASES WERE CONsistent with typical childhood ALL, indicating that identification of cases requires chromosome studies. A review of all reported cases of Ph1 + childhood ALL shows that Ph1 + patients are older and have higher initial platelet and white blood cell counts (WBC) than most children with ALL. However, a life table comparison between the reported cases of Ph1 + ALL in children and randomly selected age-, sex-, and WBC-matched controls with ALL shows the duration of first marrow remission to be significantly shorter (p less than 0.02) for the Ph1 + cases Ph1 + ALL is a distinct subtype of childhood ALL that is not rare and can be identified only by cytogenetic studies. The prognosis is poor. Cytogenetic studies should be done prospectively in a large group of children with ALL to define further the subgroup of patients and to confirm the findings of this retrospective analysis.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002904:Chromosomes, Human, 21-22 and Y; D005260:Female; D006801:Humans; D007621:Karyotyping; D007945:Leukemia, Lymphoid / Q000145:classification / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies