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Malignancies and Lymphoproliferations in Children With Primary Immune Deficiency-A Single-center Experience.
Aydogmus, Cigdem; Turkyilmaz Ucar, Ozge; Kaplan Sarikavak, Sibel; Cipe, Funda; Ulas, Selami; Turan, Isilay; Naiboglu, Sezin; Yildirim, Ilke; Kalay, Gulsah; Kapci, Nermin; Al, Serdar; Gokmirza Ozdemir, Pinar; Celiksoy, Mehmet Halil.
Affiliation
  • Aydogmus C; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Turkyilmaz Ucar O; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Kaplan Sarikavak S; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Cipe F; Department of Pediatric Allergy and Clinical Immunology, Altinbas University School of Medicine, Istanbul, Turkey.
  • Ulas S; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Turan I; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Naiboglu S; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Yildirim I; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Kalay G; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Kapci N; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Al S; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Gokmirza Ozdemir P; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
  • Celiksoy MH; Department of Pediatric Allergy and Clinical Immunology, Basaksehir Cam and Sakura City Hospital.
J Pediatr Hematol Oncol ; 46(2): e169-e173, 2024 03 01.
Article in En | MEDLINE | ID: mdl-38277623
ABSTRACT
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.
Subject(s)

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Ataxia Telangiectasia / Epstein-Barr Virus Infections / Immunologic Deficiency Syndromes / Neoplasms Type of study: Screening_studies Limits: Child / Humans Language: En Journal: J Pediatr Hematol Oncol Journal subject: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Year: 2024 Type: Article

Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Ataxia Telangiectasia / Epstein-Barr Virus Infections / Immunologic Deficiency Syndromes / Neoplasms Type of study: Screening_studies Limits: Child / Humans Language: En Journal: J Pediatr Hematol Oncol Journal subject: HEMATOLOGIA / NEOPLASIAS / PEDIATRIA Year: 2024 Type: Article