Childhood lymphomas

Abstract

If environmental factors are important in the aetiology of the major lymphomas, then much is to be gained from evaluating this group of disorders in children. There are several reasons for this. In most of the world, children are exposed to less than adults ; their environment is relatively confined and therefore more definable, quantitatively and qualitatively. It should also be appreciated that there is an adult counterpart to Hodgkin’s disease, lymphosarcoma and reticulum cell sarcoma in childhood. Putting aside the question whether each of these disorders is similar aetiologically in both age groups, intensive investigation of the lymphomas in the young can provide us with some facts relevant to older age groups. The study of childhood lymphomas as a group also affords the opportunity to evaluate the possible role of chromosomal and/or inborn immunologic defects as predisposing factors. This approach has already produced many dividends and as our ability to detect more subtle congenital defects improves, additional information of great importance will undoubtedly be gained. We know of the association between maternal radiation (Stewart and Kneale, 1970), mongolism, Kleinfelter’s syndrome and various autosomal recessive defects (Fraumeni, 1969) and certain types of leukaemia. We have also learned that certain immunologic deficiencies (the Wiskott-Aldrich syndrome, Chediak-Higashi syndrome, ataxia-telangiectasia) predispose to the lymphomas (Miller, 1968). We must now ask what other disorders, in addition to those which are readily apparent clinically, might increase ones risk of developing a lymphoreticular malignancy? Another reason for studying this group separately is that they have certain distinctive epidemiologic features which set them apart both from other malignant diseases in the young and from similar disorders in the old. But perhaps the most important reason for devoting all our efforts to this group of diseases is that they are a major cause of morbidity and mortality in children, and as such they must be viewed as tragic disorders. Although major advances have been made in therapy, our goal must still be to prevent these disorders. This can be accomplished only by identifying predisposing factors and interrupting the chain of events which ultimately leads to the development of these disorders. As a starting point, however, one must look for differences in lymphoma patterns.