Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience
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Tarih
2024
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
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.
Açıklama
Anahtar Kelimeler
Primary immune deficiencies (PIDs), Genetic disorders, İmmune function
Kaynak
Journal of Pediatric Hematology/Oncology
WoS Q Değeri
Q3
Scopus Q Değeri
Q3
Cilt
46
Sayı
2
Künye
Aydogmus, C., Ucar, O. T., Sarikavak, S. K., Cipe, F., Ulas, S., Turan, I., Naiboglu, S., Turan, I., Kalay, G., Yildirim, I., Kapci, N., Al, S., Gokmirza Ozdemir, P., Celiksoy, M. H. (2024). Malignancies and lymphoproliferations in children with primary immune deficiency-a single-center experience. Journal of Pediatric Hematology/Oncology, 46(2), e169-e173. 10.1097/MPH.0000000000002817
