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.