Immunodeficiency Diseases and Malignancy

  • Beatrice D. Spector
  • John H. Kersey
  • Robert A. Good
Part of the Sloan-Kettering Institute Cancer Series book series (SKICS)


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).


Pernicious Anemia Primary Immunodeficiency Severe Combine Immunodeficiency Primary Immunodeficiency Disease Chronic Lymphatic Leukemia 
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  1. 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.CrossRefGoogle Scholar
  2. Bergsma, D. (ed.), 1975, Immunodeficiency in Man and Animals, Birth Defects: Orig. Artic. Ser. 11, Sinauer Associates, Sunderland, Massachusetts.Google Scholar
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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
  9. Creagan, E. T., and Fraumeni, J. R., Jr., 1973, Familial gastric cancer and immunologic abnormalities, Cancer 32: 1325–1331.PubMedCrossRefGoogle Scholar
  10. 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
  11. 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
  12. 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
  13. Forssman, O., and Herner, B., 1964, Acquired agammaglobuilinemia and malabsorption, Acta Med. Scared. 176: 779–786.Google Scholar
  14. 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
  15. Fraumeni, J. F., Jr., and Miller, R. W., 1967, Epidemiology of human leukemia: Recent observations, J. Natl. Cancer Inst. 38: 593–605.Google Scholar
  16. 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
  17. 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
  18. 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
  19. Gatti, R. A., and Good, R. A., 1971, Occurrence of malignancy in immunedeficiency diseases, Cancer 28: 89–98.PubMedCrossRefGoogle Scholar
  20. Gershwin, M. E., and Steinberg, A. D., 1973, Loss of suppressor function as a cause of lymphoid malignancy, Lancet,2: 1174–1176.PubMedCrossRefGoogle Scholar
  21. Good, R. A., 1972, Relations between immunity and malignancy, Proc. Natl. Acad. Sci. U.S.A. 69: 1026–1032.Google Scholar
  22. 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
  23. 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
  24. 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
  25. 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
  26. 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
  27. 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
  28. 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
  29. Hoover, R., and Fraumeni, J. F., Jr., 1973, Risk of cancer in renal-transplant recipients, Lancet,2: 55–57.PubMedCrossRefGoogle Scholar
  30. 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
  31. 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
  32. 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
  33. 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
  34. 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
  35. 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
  36. Kersey, J. H., Spector, B. D., and Good, R. A., 1974, Cancer in children with primary immunodeficiency diseases, J. Pediatr 84: 263–264.PubMedCrossRefGoogle Scholar
  37. 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
  38. Leikola, J., Koistinen, J., Lehtinen, J., and Virolainen, M., 1973, IgA-induced anaphylactic transfusion reactions: A report of 4 cases, Blood 42: 111–119.PubMedGoogle Scholar
  39. 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
  40. Lukes, R. J., and Collins, R. D., 1974, Immunologic characteristics of malignant lymphomas, Cancer 34 (Suppl. 4): 1488–1503.PubMedCrossRefGoogle Scholar
  41. Manolova, G., and Manolova, Y., 1972, Marker band in one chromosome 14 from Burkitt’s lymphomas, Nature (London) 237: 33–34.CrossRefGoogle Scholar
  42. 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
  43. Medical Research Council, 1970, Hypogammaglobulinemia in the United Kingdom, Her Majesty’s Stationery Office, London.Google Scholar
  44. 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
  45. Miller, R. W., 1969, Fifty two forms of childhood cancer: United States mortality experience, 1960–1966, J. Pediatr. 75: 685–689.PubMedCrossRefGoogle Scholar
  46. 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
  47. Morell, A., 1973, personal communication to the Immunodeficiency-Cancer Registry.Google Scholar
  48. Morgan, J. L., Holcomb, T. M., and Morrissey, R. W., 1968, Radiation reaction in ataxiatelangiectasia, Am. J. Dis. Child. 116: 537–538.Google Scholar
  49. Ochs, H., 1973, personal communication to the Immunodeficiency-Cancer Registry.Google Scholar
  50. 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.PubMedCrossRefGoogle Scholar
  51. 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.CrossRefGoogle Scholar
  52. Penn, I., 1975, The incidence of malignancies in transplant recipients, Transplant. Proc. 7:323.PubMedGoogle Scholar
  53. 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. PubMedGoogle Scholar
  54. 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
  55. 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
  56. 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
  57. 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
  58. 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
  59. Southam, C. J., 1961, Application of immunology to clinical cancer: Past attempts and future possibilities, Cancer Res. 21: 1302–1361.Google Scholar
  60. Spector, B. D., Kersey, J. H., and Perry, G. S., III, 1978, Immunodeficiency cancer registry (in preparation).Google Scholar
  61. 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.PubMedGoogle Scholar
  62. 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
  63. 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
  64. Walker, W. A., and Hong, R., 1973, Immunology of the gastrointestinal tract, Part I, J. Pediatr. 83: 517–530.Google Scholar
  65. 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
  66. Williams, W. J., Beutler, E., Erslev, A., and Rundles, R. W. (eds.), 1972, Hematology, McGraw-Hill, New York.Google Scholar
  67. 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.CrossRefGoogle Scholar
  68. Young, J. L., and Miller, R. W., 1975, Incidence of malignant tumors in U.S. children, J. Pediatr. 86: 254–258.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1978

Authors and Affiliations

  • Beatrice D. Spector
    • 1
  • John H. Kersey
    • 1
  • Robert A. Good
    • 2
  1. 1.Department of Laboratory Medicine and PathologyUniversity of MinnesotaMinneapolisUSA
  2. 2.Memorial Sloan-Kettering Cancer CenterNew YorkUSA

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