Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry.
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2018-03
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Abstract
Background
We evaluated the overall and site-specific incidence of cancer in subjects with primary immunodeficiency diseases (PIDD) enrolled in the United States Immune Deficiency Network (USIDNET) registry compared with age-adjusted cancer incidence in the Surveillance, Epidemiology and End Results Program (SEER) database.Objective
We hypothesized that subjects with PIDD would have an increased incidence of cancer due to impaired immune function.Methods
Overall and site-specific cancer incidence rates were evaluated in subjects with PIDD (n = 3658) enrolled in the USIDNET registry from 2003 to 2015 and compared with age-adjusted incidence rates in the SEER database.Results
We observed a 1.42-fold excess relative risk of cancer in subjects with PIDD compared with the age-adjusted SEER population (P < .001). Men with PIDD had a 1.91-fold excess relative risk of cancer compared with the age-adjusted male population (P < .001), while women with PIDD had similar overall cancer rates compared with the age-adjusted female population. Of the 4 most common malignancies in men and women in SEER (lung, colon, breast, and prostate cancers), we found no significant increase in these diagnoses in subjects with PIDD. Significant increases in lymphoma in both men (10-fold increase, P < .001) and women (8.34-fold increase, P < .001) with PIDD were observed.Conclusions
Excess incidence of cancer occurred in subjects with PIDD. An excess of lymphoma in specific PIDD populations principally drove this increased incidence, while no increased risk of the most common solid tumor malignancies was observed. These data point to a restricted role of the immune system in protecting from specific cancers.Type
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Mayor, Paul C, Kevin H Eng, Kelly L Singel, Scott I Abrams, Kunle Odunsi, Kirsten B Moysich, Ramsay Fuleihan, Elizabeth Garabedian, et al. (2018). Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry. The Journal of allergy and clinical immunology, 141(3). pp. 1028–1035. 10.1016/j.jaci.2017.05.024 Retrieved from https://hdl.handle.net/10161/32137.
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Scholars@Duke
Patricia Lynne Lugar
I have a strong interest and research background in the study of dysregulated B lymphocytes, B cells, that characterize clinical disease such as autoimmune disease and humoral immune deficiency. My current research projects are aimed at the study of humoral immune deficiency, specifically common variable immune deficiency or CVID and the dysregulation of B cells in the generation of lymphoma and autoimmune disease.
Other ongoing research projects include characterizing the molecular and cellular events that cause a common disease, chronic idiopathic urticaria or CIU and rare disorders idiopathic anaphylaxis and idiopathic angioedema.
Rebecca Hatcher Buckley
The overall emphasis of Dr. Buckley's research is in human T,B and NK cell development and in aberrations in their development and regulation. The work involves three particular areas of investigation: 1) the cellular and molecular bases of genetically-determined human immunodeficiency diseases, 2) the use of bone marrow stem cells to cure genetically-determined immunodeficiency diseases, and 3) the use of human SCID bone marrow stem cell chimeras to study human thymic education, T and B cell ontogeny, tolerance induction and MHC restriction mechanisms. Methodology includes monoclonal antibody (mAb) analyses of lymphocyte phenotypes, a variety of T cell and natural killer (NK) cell functional assays, studies of thymic output by T cell receptor recombination excision circle measurement, studies of T cell diversity by spectratyping, studies of T cell longevity by telomere analysis and assessment of B cell differentiation and function. A unique resource available for her studies is the largest population of patients with genetically-determined immunodeficiency diseases in the U.S., which includes the largest population in the world of longterm SCID chimeras treated at a single center, some of whom have been studied and followed for more than 37 years. The administration of rigorously T cell depleted haploidentical bone marrow stem cells to SCID recipients without pre-transplant conditioning or post-transplant use of immunosuppressive drugs to prevent GVHD provides an unmanipulated system for studying human thymic education, T and B cell ontogeny, MHC restriction mechanisms and tolerance induction. Studies to identify mutations in patients with primary immunodeficiency are continuing, particularly in those with SCID.
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