Immunoreconstitution by Bone Marrow Transplantation Decreases Lymphoproliferative Malignancies in Wiskott-Aldrich and Severe Combined Immune Deficiency Syndromes
Data linking immunodeficient individuals to a high incidence of malignancy has been collected through the analysis of outcomes in renal allograft recipients (1) and patients with naturally occurring immunodeficiency states such as severe combined immune deficiency (SCID), Wiskott-Aldrich syndr ome (WAS) and ataxia telangiectasia (2–4). The mechanisms involved in the pathogenesis of malignancy in patients with naturally occurring or acquired immunodeficiencies are poorly understood. In large part this is due to the heterogeneity in the scope and severity of immune deficiency in these patients. In addition, some patients are exposed to potentially oncogenic agents, such as Epstein-Barr virus (EBV), that may influence the development of malignancy. Despite the complexity of these patients, certain aspects of the relationship between naturally occurring immune deficiency and malignancy can be studied. Many patients with SCID and WAS have been successfully immunoreconstituted by bone marrow transplantation (BMT). Therefore, it has become possible to ask whether immunoreconstitution prevents the development of malignancy in these patients. This chapter addresses this question by analyzing cases of malignancy in immunodeficient individuals that have undergone either BMT or thymus transplantation. These data will be compared to previously reported cases of malignancies in untransplanted immunodeficient patients.
KeywordsPost Transplant Ataxia Telangiectasia Severe Combine Immunodeficiency Severe Combine Immune Deficiency Renal Allograft Recipient
Unable to display preview. Download preview PDF.
- 1.Penn, I. Malignancies associated with immunosuppressive or cytotoxic therapy. Surgery, 83:294, 1978.Google Scholar
- 3.Filipovich, A.H., Zerbe, D., Spector, B., and Kersey, J. Lymphomas in persons with naturally occurring immunodeficiency disorders. In: I.T. Magrath (ed.), The Influence of the Environment on Leukemia and Lymphoma Subtypes. New York: Raven Press, 1983.Google Scholar
- 9.Kapoor, N., Kirkpatrick, D., Blaese, R., Oleske, J., Hilgartner, M., Changanti, R., Good, R., and O’Reilly, R. Reconstitution of normal megakaryocytopoiesis and immunologic functions in Wiskott-Aldrich syndrome by marrow transplantation following myeloablation and immunosuppression with busulfan and cyclophosphamide. Blood, 57:692, 1981.PubMedGoogle Scholar
- 15.Thestrup-Pedersen, K., Esmann, V., Jensen, J.R., Hastrup, J., Thorling, K., Saemundsen, A.K., Bisballe, S., Oallesen, G., Madsen, M., Grazia-Masucci, M., and Emberg, I. Epstein-Barr virus-induced lymphoproliferative disorder converting to fatal Burkitt-like lymphoma in a boy with interferon inducible chromosomal defect. Lancet, ii:997, 1980.CrossRefGoogle Scholar
- 17.Filipovich, A.H. and Spector, B.D. Immunodeficiency (ID) as a susceptibility factor for malignancy in ataxia telangiectasia (AT): report from the Immunodeficiency-Cancer Registry (ICR). Pediat. Res., 15(4):806, 1981.Google Scholar