Immunodeficiency syndromes. X-linked agammaglobulinemia, common variable immunodeficiency, Chédiak-Higashi syndrome, Wiskott-Aldrich syndrome, and X-linked lymphoproliferative disorder

Abstract

The incidence and mortality of neoplasia in patients with primary immunodeficiencies exceed the anticipated rates in the normal population by approximately 100 to 300 times. Lymphoreticular malignancies account for the majority of tumors, although solid tumors, especially gastric carcinoma, occur with increased frequency as well. More than half of these neoplasms are diagnosed by ten years of age, except in patients with a later age of onset of immunodeficiency. Five primary immunodeficiency disorders with an increased risk of neoplasia are reviewed.