Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome.

The results of a nationwide survey on primary immunodeficiency syndrome (PIS) in Japan are presented. By the repeated questionnaire method, 497 PIS cases were collected prior to February 1979 with clinical information. Numbers of each type of PIS, age at the time of diagnosis, patient's status at the time of registration, familial incidence of PIS, and development of malignancy, autoimmune diseases, and allergic diseases among all reported patients are presented and discussed.