Abstract
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.
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Neudorf, S.M.L., Filipovich, A.H., Kersey, J.H. (1984). Immunoreconstitution by Bone Marrow Transplantation Decreases Lymphoproliferative Malignancies in Wiskott-Aldrich and Severe Combined Immune Deficiency Syndromes. In: Purtilo, D.T. (eds) Immune Deficiency and Cancer. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-4760-6_24
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DOI: https://doi.org/10.1007/978-1-4684-4760-6_24
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