Chronic myelogenous leukemia in childhood

Abstract

The authors present 4 children with the infantile form and 2 cases with the juvenile form of chronic myelogenous leukemia. (This terminology is confusing as there are other authors, who use the terms juvenile for infantile and adult for juvenile, respectively). In their comments the authors present a theory explaining the finding of fetal Hb and the absence of Ph1 chromosome in the infantile form whereas in the juvenile form the amount of fetal Hb is normal and the Ph1 chromosome is recovered in the blood cells of the patients. When the Ph1 chromosome is found the beta chain globin synthesis is normal. When the autosome 22 is not visibly altered, beta chain globin synthesis is suppressed and fetal Hb appears. Thus there should be a relationship between the chromosome 22 and the regulation of the type of Hb. The production of either fetal Hb or an alteration of the autosome 22 may be related to the time of exposure of the cell to an environmental influence. When the exposure happens at an early stage of gametogenesis chromosome 22 is altered functionally; when the noxious agent acts postzygotically the Ph1 chromosome is produced. Thus the infantile and juvenile forms of CML are different modes of presentation of a similar disease. The difference between the two types is due to the action of an unknown noxious agent at different stages of embryonal development. (Wegelius - Helsinki)