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In this retrospective, single\u2010center study of 647 CVID subjects followed over 4 decades, we present immunologic and clinical phenotypes, pathology, treatment, and outcomes of 45 patients (15 males and 30 females, 7%) who developed 49 lymphoid malignancies. The mean age at CVID diagnosis was 42.6\u00a0years) and at lymphoma diagnosis was 48.8\u00a0years. Of the 41 with known follow up, 29 (70%) have died, 27 of these due to this diagnosis. Twelve are alive, in remission or have achieved cure; four others were alive at last encounter. Some patients had a history of only recurrent infections (36.3%); others had autoimmunity (33%), enteropathy (20%), and\/or granulomatous disease (11%). Six had previously been treated for another cancer. This report also includes 6 additional living CVID patients who had been diagnosed with NHL; 4 were given treatment for this. However, on pathology review, the initial diagnosis was reversed, as the findings were more consistent with a benign lymphoproliferative process. This study outlines the high incidence of lymphoma in this single CVID cohort, and some of the diagnostic challenges presented due to immune dysregulation characteristic of this immune defect.<\/jats:p>","DOI":"10.1111\/ejh.13687","type":"journal-article","created":{"date-parts":[[2021,7,13]],"date-time":"2021-07-13T15:02:35Z","timestamp":1626188555000},"page":"503-516","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Lymphoid malignancy in common variable immunodeficiency in a single\u2010center cohort"],"prefix":"10.1111","volume":"107","author":[{"given":"Tukisa","family":"Smith","sequence":"first","affiliation":[{"name":"Division of Clinical Immunology Departments of Medicine and Pediatrics PRISM Immunology Institute Icahn School of Medicine at Mount Sinai New York NY USA"},{"name":"Allergy and Immunology Department of Medicine UC San Diego Health San Diego CA 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