Malignancies and Lymphoproliferations in Children With Primary Immune Deficiency—A Single-center Experience

恶性肿瘤 医学 免疫失调 免疫系统 淋巴增殖性病變 免疫学 原发性免疫缺陷 人口 淋巴瘤 爱泼斯坦-巴尔病毒 病毒 内科学 环境卫生
作者
Çiğdem Aydoğmuş,Ö. Uçar,sibel kaplan sarikavak,Funda Çipe,Selami Ulas,Işilay Turan,Sezin Naiboğlu,İlke Yıldırım,Gülşah Kalay,Nermin Kapçı,Serdar Al,Pınar Gökmirza Özdemir,Mehmet Halil Çelıksoy
出处
期刊:Journal of Pediatric Hematology Oncology [Ovid Technologies (Wolters Kluwer)]
卷期号:46 (2): e169-e173
标识
DOI:10.1097/mph.0000000000002817
摘要

Primary immune deficiencies (PIDs) are rare genetic disorders characterized by impaired immune function, leading to frequent infections and immune dysregulation. Studies have shown that individuals with PID are at an increased risk of developing malignancies and lymphoproliferative disorders compared with the general population. In this single-center study, we aimed to analyze the occurrence of malignancies and lymphoproliferations in children diagnosed with PID. We retrospectively analyzed the medical records of 550 pediatric patients diagnosed with PIDs at our center. Among them, 17 (3,0%) patients were identified with malignancy and/or benign lymphoproliferation. Eight of the 17 patients (47.0%) had immune dysregulatory diseases, whereas ataxia-telangiectasia was the second most common PID associated with malignancy and/or benign lymphoproliferation (n = 5, 29.4%). Lymphoma was the predominant malignancy (n = 11, 64.7%), and Epstein-Barr virus was identified as the most common viral agent associated with malignancy and/or benign lymphoproliferation in patients with PID (n = 8, 47.0%). Our study highlights the association between PID and malignancies/lymphoproliferations, with immune dysregulation syndromes being the most common subclass associated with malignancies/lymphoproliferations. Early diagnosis, multidisciplinary management, and regular surveillance are crucial in improving patient outcomes and saving lives.
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