Malignant neoplasms in registered cases of primary immunodeficiency syndrome.
The occurrence of malignant neoplasms among registered cases of primary immunodeficiency syndrome was studied. A total of 17 malignant tumors occurred in 596 (2.9%) infant patients; two in eight (25%) patients with Chediak-Higashi syndrome, seven in 49 (14.3%) with ataxia-telangiectasia and one in 12 (8.3%) patients with immunodeficiency with hyper-immunoglobulin-M-emia. The overall incidence of malignant tumors in these cases was 200-300 times that in the general population. The incidence in Japan was compared with that in the U.S.A. and in Italy.
D000293:Adolescent; D000328:Adult; D001260:Ataxia Telangiectasia / Q000150:complications; D002609:Chediak-Higashi Syndrome / Q000150:complications; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D007223:Infant; D007558:Italy; D007564:Japan; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000453:epidemiology* / Q000209:etiology; D012042:Registries