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Primary immunodeficiency diseases are associated with an increased tendency for noninfectious complications of autoimmunity and malignancy, particularly leukemia and lymphoma. The mechanisms of immune dysregulation have been linked to the combination of dysregulated immune cells and environmental factors such as infections. In particular, dysfunction in T-cell subsets and Epstein-Barr virus contributes to the development of autoimmunity and lymphoproliferative disease in primary immunodeficiency diseases. There are scant reports of patients with partial DiGeorge syndrome and Epstein-Barr virus-driven lymphoma. We report 1 patients with partial DiGeorge syndrome who developed lymphoma, and review reported cases in the literature. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

Citation

Michell Lozano-Chinga, Natalie Diaz-Cabrera, Farhad Khimani, Karin Chen, John Bohnsack, Jolan E Walter, Farnaz Tabatabaian, Zeinab Afify. Lymphoma in Partial DiGeorge Syndrome: Report of 2 Cases. Journal of pediatric hematology/oncology. 2022 Apr 01;44(3):e819-e822

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PMID: 34966099

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