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Lymphoid malignancy in common variable immunodeficiency in a single-center cohort.


ABSTRACT: One of the complications of common variable immunodeficiency (CVID) is the development of lymphoid malignancy. In this retrospective, single-center 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 years) and at lymphoma diagnosis was 48.8 years. 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

SUBMITTER: Smith T 

PROVIDER: S-EPMC8497444 | biostudies-literature | 2021 Nov

REPOSITORIES: biostudies-literature