Abstract
This review of immunodeficiency diseases and malignancy is based primarily on evidence provided by over 200 cases of cancer occurring in patients with diagnosed primary immunodeficiency diseases. The cases were collected by the Immunodeficiency-Cancer Registry, a tumor registry established in 1971 under the aegis of the World Health Organization Committee on Primary Immunodeficiencies (Fudenberg et al., 1971). Compilation of case reports provides evidence that immunodeficiency diseases predispose to malignancy, since mortality rates for cancer in immunodeficiency groups exceed by 100 times the expected rates for the general population (Kersey et al., 1974). An intimate association between an abnormal immune system and subsequent development of malignancy also exists among renal transplant recipients on immunosuppressive regimens, who have developed lymphomas at significantly higher rates than expected for an age-and sex-matched population during a comparable period (Penn, 1975; Hoover and Fraumeni, 1973). Another condition, the actual presence of a malignancy, demonstrates the effect of cancer on the immune system in persons with Hodgkin’s disease, chronic lymphatic leukemia, and multiple myeloma (Schier, 1954; Kelly et al., 1958; Southam, 1961; Cone and Uhr, 1964; Dent et al., 1968).
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Spector, B.D., Kersey, J.H., Good, R.A. (1978). Immunodeficiency Diseases and Malignancy. In: Lipkin, M., Good, R.A. (eds) Gastrointestinal Tract Cancer. Sloan-Kettering Institute Cancer Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-2442-3_3
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