Primary immunodeficiency syndromes and malignant neoplasms

Among 727 patients registered to the Japan Immunodeficiency Registry up to December 6, 1986 from 1975, 25 patients were reported to have developed malignant neoplasms. The incidence of malignant neoplasms in 649 childhood patients was 3.2%, which was approximately 400 times higher than general childhood populations. More than half of these patients were complicated with malignant limphomas. The patients with Chediak-Higashi syndrome and ataxia-telangiectasia showed the highest incidence of developing malignant neoplasms.