Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Utah.
Division of Allergy and Immunology, Department of Internal Medicine, University of South Florida Morsani College of Medicine.
J Pediatr Hematol Oncol. 2022 Apr 1;44(3):e819-e822. doi: 10.1097/MPH.0000000000002388.
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.
原发性免疫缺陷病与非传染性自身免疫和恶性肿瘤并发症（尤其是白血病和淋巴瘤）的发生风险增加相关。免疫失调的机制与失调的免疫细胞和环境因素（如感染）的组合有关。特别是，T 细胞亚群和 Epstein-Barr 病毒功能障碍导致原发性免疫缺陷病中自身免疫和淋巴增生性疾病的发展。关于部分 DiGeorge 综合征和 Epstein-Barr 病毒驱动的淋巴瘤患者的报道很少。我们报告了 1 例部分 DiGeorge 综合征患者发生淋巴瘤，并复习了文献中的报道病例。
