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Immunodeficiency Diseases and Malignancy

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Gastrointestinal Tract Cancer

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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References

  • Amman, A. J., and Hong, R., 1971, Selective IgA deficiency: Presentation of 30 cases and a review of the literature, Medicine (Baltimore) 50: 223–236.

    Article  Google Scholar 

  • Bergsma, D. (ed.), 1975, Immunodeficiency in Man and Animals, Birth Defects: Orig. Artic. Ser. 11, Sinauer Associates, Sunderland, Massachusetts.

    Google Scholar 

  • Blaese, R. M., Strober, W., and Waldmann, T. A., 1975, Immunodeficiency in the WiskottAldrich syndrome, in: Immunodeficiency in Man and Animals (D. Bergsma, ed.), Birth Defects: Orig. Artic. Ser. 11:250–254, Sinauer Associates, Sunderland, Massachusetts.

    Google Scholar 

  • Bochkov, N. P., Lopukhin, Y. M., Kuleshov, N. P., and Kovalchuk, L. V., 1974, Cytogenetic study of patients with ataxia-telangiectasia, Humangenetik,24: 225–238.

    Google Scholar 

  • Boder, E., 1975, Ataxia-telangiectasia: Some historic, clinical, and pathologic observations, in: Immunodeficiency in Man and Animals (D. Bergsma, ed.), Birth Defects: Orig. Artic. Ser. 11: 255270, Sinauer Associates, Sunderland, Massachusetts.

    Google Scholar 

  • Chaplin, H., 1975, personal communication to the Immunodeficiency-Cancer Registry. Cleaver, J. E., 1968, Defective repair rèplication of DNA in xeroderma pigmentosum, Nature (London), 218: 652–656.

    Google Scholar 

  • Cone, L., and Uhr, J. W., 1964, Immunological deficiency diseases associated with chronic lymphatic leukemia and multiple myeloma, J. Clin. Invest. 43: 2241–2248.

    Google Scholar 

  • Cooper, M. D., Faulk, W. P., Fudenberg, H. H., Good, R. A., Hitzig, W., Kunkel, H., Rosen, F., Seligmann, M., Soothill, J., and Wedgwood, R. J., 1973, Classification of primary immunodeficiencies, N. Engl. J. Med.288: 966–967.

    Google Scholar 

  • Creagan, E. T., and Fraumeni, J. R., Jr., 1973, Familial gastric cancer and immunologic abnormalities, Cancer 32: 1325–1331.

    Article  PubMed  CAS  Google Scholar 

  • Cunliffe, P. N., Mann, J. R., Cameron, A. H., and Roberts, K. D., 1975, Radiosensitivity in ataxia-telangiectasia, Br. J. Radiol. 48: 374–376.

    Google Scholar 

  • Cutler, S. J., and Young, J. L., Jr., 1975, Third National Cancer Survey: Incidence Data, National,Cancer Institute Monograph 41, U.S. Department of Health, Education, and Welfare, Washington, D.C.

    Google Scholar 

  • Dent, P. B., Peterson, R. D. A., and Good, R. A., 1968, The relationship between immunologic function and oncogenesis, in: Immunologic Deficiency Diseases in Man ( R. A. Good and D. Bergsma, eds.), Vol. 4, pp. 443–453, National Foundation Press, New York.

    Google Scholar 

  • Forssman, O., and Herner, B., 1964, Acquired agammaglobuilinemia and malabsorption, Acta Med. Scared. 176: 779–786.

    Google Scholar 

  • Fraser, K. J., and Rankin, J. G., 1970, Selective deficiency of IgA immunoglobulins associated with carcinoma of the stomach, Amt. Ann. Med. 19: 165–167.

    Google Scholar 

  • Fraumeni, J. F., Jr., and Miller, R. W., 1967, Epidemiology of human leukemia: Recent observations, J. Natl. Cancer Inst. 38: 593–605.

    Google Scholar 

  • Fraumeni, J. F., Jr., Manning, M. D., and Mitus, W. J. 1971, Acute childhood leukemia: Epidemiologic study by cell type of 1,263 cases at the Children’s Cancer Research Foundation in Boston, 1947–65, J. Natl. Cancer Inst. 46: 461–470.

    Google Scholar 

  • Fudenberg, H. H., Good, R. A., Goodman, H. C., Hitzig, W., Hunkel, H., Roitt, I., Rosen, F., Rowe, D., Seligmann, M., and Soothill, J., 1971, Primary immunodeficiencies: Report of a World Health Organization Committee ( Special article ), Pediatrics 47: 927–946.

    Google Scholar 

  • Gardner, S. D., Field, S. M., Coleman, D., and Hulme, B., 1971, New human papovavirus ( B.K.) isolated from urine after renal transplantation, Lancet 1: 1253–1257.

    Google Scholar 

  • Gatti, R. A., and Good, R. A., 1971, Occurrence of malignancy in immunedeficiency diseases, Cancer 28: 89–98.

    Article  PubMed  CAS  Google Scholar 

  • Gershwin, M. E., and Steinberg, A. D., 1973, Loss of suppressor function as a cause of lymphoid malignancy, Lancet,2: 1174–1176.

    Article  PubMed  CAS  Google Scholar 

  • Good, R. A., 1972, Relations between immunity and malignancy, Proc. Natl. Acad. Sci. U.S.A. 69: 1026–1032.

    Google Scholar 

  • Gotoff, S. O., Amirmokri, E., and Liebner, E. J., 1967, Ataxia-telangiectasia: Neoplasia, unto- ward response to X-irradiation and tuberous sclerosis, Am. J. Dis. Child. 114: 617–625.

    Google Scholar 

  • Grundy, G. W., Creagan, E. T., and Fraumeni, J. F., Jr., 1973, Non-Hodgkin’s lymphoma in childhood: Epidemiologic features, J. Natl. Cancer Inst. 51: 767–776.

    Google Scholar 

  • Haerer, A. F., Jackson, J. F., and Evers, C. G., 1969, Ataxia-telangiectasia with gastric carcinoma, J. Am. Med. Assoc. 210: 1884–1887.

    Google Scholar 

  • Hamoudi, A. B., Ertel, I., Newton, W. A., Jr., Reiner, C. B., and Clatworthy, H. W., 1974, Multiple neoplasms in an adolescent child associated with IgA deficiency, Cancer?33: 1134—1144.

    Google Scholar 

  • Hanson, L. A., 1975, personal communication to the Immunodeficiency-Cancer Registry. Hecht, F., McCaw, B., and Koler, R. D., 1973, Ataxia-telangiectasia: Clonal growth of translocation lymphocytes, N. Engl. J. Med. 289: 286–291.

    Google Scholar 

  • Hermans, P. E., Diaz-Buxo, J. A., and Stobo, J. D., 1976, Idiopathic late-onset immunoglobulin deficiency: Clinical observations in 50 patients, Am. J. Med. 61: 221–237.

    Google Scholar 

  • Hobbs, J., 1975, IgM deficiency, in: Immunodeficiency in Man and animals (D. Bergsma, ed.), Birth Defects: Orig. Artic. Ser. 11:112–116, Sinaur Associates, Sunderland, Massachusetts.

    Google Scholar 

  • Hoover, R., and Fraumeni, J. F., Jr., 1973, Risk of cancer in renal-transplant recipients, Lancet,2: 55–57.

    Article  PubMed  CAS  Google Scholar 

  • Jackson, J. F., 1972, Ataxia-telangiectasia, in: Skin, Heredity, and Malignant Neoplasma ( H. T. Lynch, ed.), pp. 94–103, Medical Examination Publishing Co., Flushing, New York.

    Google Scholar 

  • Kelly, W. D., Good, R. A., and Varco, R. L., 1958, Anergy and skin homograft survival in Hodgkin’s disease, Surg. Gynecol. Obstet. 107: 565–570.

    Google Scholar 

  • Kersey, J. H., and Gajl-Peczalska, K. J., 1975, T and B lymphocytes in humans: A review, Am. J. Pathol. 81: 446–458.

    Google Scholar 

  • Kersey, J. H., and Spector, B. D., 1975, Immune deficiency diseases, in: Persons at High Risk of Cancer: An Approach to Cancer Etiology and Control (J. F. Fraumeni, Jr., ed.), pp. 55–67, Academic Press, New York.

    Google Scholar 

  • Kersey, J., Spector, B. D., and Good, R. A., 1973a, Immunodeficiency and cancer, in: Advances in Cancer Research ( S. Weinhouse and G. Klein, eds.), Vol. 18, pp. 211–230, Academic Press, New York.

    Google Scholar 

  • Kersey, J. H., Spector, B. D., and Good, R. A., 1973b, Primary immunodeficiency diseases and cancer: The Immunodeficiency-Cancer Registry, Int. J. Cancer 12: 333–347.

    Google Scholar 

  • Kersey, J. H., Spector, B. D., and Good, R. A., 1974, Cancer in children with primary immunodeficiency diseases, J. Pediatr 84: 263–264.

    Article  PubMed  CAS  Google Scholar 

  • Kirkpatrick, C., 1976, personal communication to the Immunodeficiency-Cancer Registry. Krivit, W., and Good, R. A., 1959, Aldrich’s syndrome (thrombocytopenia, eczema, and infection in infants), AMA J. Dis. Child. 97: 137–153.

    Google Scholar 

  • Leikola, J., Koistinen, J., Lehtinen, J., and Virolainen, M., 1973, IgA-induced anaphylactic transfusion reactions: A report of 4 cases, Blood 42: 111–119.

    PubMed  CAS  Google Scholar 

  • Levin, S., and Perlov, S., 1971, Ataxia-telangiectasia in Isreal with observations on its relationship to malignant disease, Isr. J. Med. Sci. 7: 1535–1541.

    Google Scholar 

  • Lukes, R. J., and Collins, R. D., 1974, Immunologic characteristics of malignant lymphomas, Cancer 34 (Suppl. 4): 1488–1503.

    Article  PubMed  Google Scholar 

  • Manolova, G., and Manolova, Y., 1972, Marker band in one chromosome 14 from Burkitt’s lymphomas, Nature (London) 237: 33–34.

    Article  Google Scholar 

  • McCaw, B. K., Hecht, F., Harnden, D. G., and Teplitz, R., 1975, Somatic rearrangement of chromosome 14 in human lymphocytes, Proc. Natl. Acad. Sci. U.S.A. 72: 2071–2075.

    Google Scholar 

  • Medical Research Council, 1970, Hypogammaglobulinemia in the United Kingdom, Her Majesty’s Stationery Office, London.

    Google Scholar 

  • Melief, C. J. M., and Schwartz, R. S., 1975, Immunocompetence and malignancy, in: Cancer: A Comprehensive Treatise (F. F. Becher, ed.), Vol. 1, pp. 121–160, Plenum Press, New York. Miller, R. W., 1967, Persons at exceptionally high risk of leukemia, Cancer Res. 27: 2420–2423.

    Google Scholar 

  • Miller, R. W., 1969, Fifty two forms of childhood cancer: United States mortality experience, 1960–1966, J. Pediatr. 75: 685–689.

    Article  PubMed  CAS  Google Scholar 

  • Miller, W. V., Holland, P. V., Sugarbaker, E., Strober, W., and Waldmann, T. A., 1970, Anaphylactic reactions to IgA: A difficult transfusion problem, Am. J. Clin. Pathol. 54: 618621.

    Google Scholar 

  • Morell, A., 1973, personal communication to the Immunodeficiency-Cancer Registry.

    Google Scholar 

  • Morgan, J. L., Holcomb, T. M., and Morrissey, R. W., 1968, Radiation reaction in ataxiatelangiectasia, Am. J. Dis. Child. 116: 537–538.

    Google Scholar 

  • Ochs, H., 1973, personal communication to the Immunodeficiency-Cancer Registry.

    Google Scholar 

  • Padgett, B. L., Walker, D. L., Zurhein, G. M., and Eckroade, R. J., 1971, Cultivation of papova- like virus from human brain with progressive multifocal leucoencephalopathy, Lancet 1: 1257–1260.

    Article  PubMed  CAS  Google Scholar 

  • Paterson, M. C., Smith, B. P., Lohman, P. H. M., Anderson, A. K., and Fishman, L., 1976, Defective excision repair of gamma ray-damaged DNA in human (ataxia-telangiectasia) fibroblasts, Nature (London) 260: 444–446.

    Article  CAS  Google Scholar 

  • Penn, I., 1975, The incidence of malignancies in transplant recipients, Transplant. Proc. 7:323.

    PubMed  CAS  Google Scholar 

  • Petit, P., Verhest, A., Lecluse van der Bilt, F., and Jengsma, A., 1972, The chromosomes of the EB virus-positive Burkitt cell line PsJ.HRJK studied by the fluorescent staining technique, Pathol. Eur. 7: 17–21.

    PubMed  CAS  Google Scholar 

  • Rees Jones, A., 1976, personal communication to the Immunodeficiency-Cancer Registry. Reeves, B. R., 1973, Cytogenetics of malignant lymphomas: Studies using a Giemsa-banding technique, Humangenetik 20: 231–250.

    Google Scholar 

  • Rolles, T. E., 1973, personal communication to the Immunodeficiency-Cancer Registry. Schier, W. W., 1954, Cutaneous anergy and Hodgkin’s disease, N. Engl. J. Med. 250: 353–361.

    Google Scholar 

  • Schuler, D., Schöngut, L., Cserhati, E., Siegler, J., and Gacs, G., 1971, Lymphoblastic transforma- tion, chromosome pattern and delayed-type skin reaction in ataxia-telangiectasia, Acta Paediatr. Scand. 60: 66–72.

    Google Scholar 

  • Sedgwick, R. P., and Boder, E., 1972, Ataxia-telangiectasia, in: Handbook of Clinical Neurology ( P. J. Vinken and G. W. Bruyen, eds.), pp. 267–339, North-Holland, Amsterdam.

    Google Scholar 

  • Shackelford, G. D., and McAlister, W. H., 1975, Primary immunodeficiency disease and ma- lignancy, Am. J. Roentgenol. Radium Ther. Nucl. Med. 123: 144–153.

    Google Scholar 

  • Southam, C. J., 1961, Application of immunology to clinical cancer: Past attempts and future possibilities, Cancer Res. 21: 1302–1361.

    Google Scholar 

  • Spector, B. D., Kersey, J. H., and Perry, G. S., III, 1978, Immunodeficiency cancer registry (in preparation).

    Google Scholar 

  • Swift, M., Sholman, L., Perry, M., and Chase, C., 1976, Malignant neoplasma in the families of patients with ataxia-telangiectasia, Cancer Res. 36: 209–215.

    PubMed  CAS  Google Scholar 

  • Takemoto, K. K., Rabson, A. S., Mullarkey, M. F., Blaese, R. M., Garon, C. F., and Nelson, D., 1974, Isolation of papovavirus from brain tumor and urine of a patient with Wiskott-Aldrich syndrome, J. Natl. Cancer Inst. 53: 1205–1207.

    Google Scholar 

  • Twomey, J. J., Jordan, P. H., Laughter, A. H., Meuwissen, H. J., and Good, R. A., 1970, The gastric disorder in immunoglobulin patients, Ann. Intern. Med. 72: 499–504.

    Google Scholar 

  • Walker, W. A., and Hong, R., 1973, Immunology of the gastrointestinal tract, Part I, J. Pediatr. 83: 517–530.

    Google Scholar 

  • Weiner, L. P., Herndon, R. M., Narayan, O., Johnson, R. T., Shar, K., Rubinstein, L. J., Preziosk, T. J., and Conley, F. K., 1972, Isolation of virus related to SV40 from patients with progressive multifocal leukoencephalopathy, N. Engl. J. Med. 286: 385–390.

    Google Scholar 

  • Williams, W. J., Beutler, E., Erslev, A., and Rundles, R. W. (eds.), 1972, Hematology, McGraw-Hill, New York.

    Google Scholar 

  • Wurster-Hill, D. H., McIntyre, O. R., Cornell, G. G., and Maurer, L. H., 1973, Marker- chromosome 14 in multiple myeloma and plasma-cell leukaemia, Lancet 2: 1031.

    Article  Google Scholar 

  • Young, J. L., and Miller, R. W., 1975, Incidence of malignant tumors in U.S. children, J. Pediatr. 86: 254–258.

    Article  PubMed  Google Scholar 

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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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  • DOI: https://doi.org/10.1007/978-1-4684-2442-3_3

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