Rejin Kebudi*, Ayca Kiykim* and Merve Karaca Sahin Pages 1 - 6 ( 6 )
The life span of patients with primary and secondary immunodeficiency has increased due to recent advances in diagnostic and therapeutic strategies. Primary immune deficiencies (PIDs) are genetic disorders that predispose patients to frequent infections, autoimmunity and malignancies. Genomic instability due to defective DNA repair processes and other unknown mechanisms in PID patients leads to an enhanced risk of cancer. PIDs were originally described as rare, to only occur in infants and young children, and to be associated with severe clinical symptoms. However, advances in gene sequencing technologies, have revealed that they are much more common than originally appreciated and are present in older children, adolescents, and adults. After infection, malignancy is the most prevalent cause of death in both children and adults with PIDs. The overall risk for developing cancer in patients with PID is estimated to range from 4.7 to 5.7 percent. A 1.4 to 1.6-fold excess relative risk of cancer has been reported for PIDs. In this paper, we review the various categories of PIDs in children and highlight their association with various malignancies. MEDLINE was searched to identify articles for inclusion. Three authors have independently screened literature search results and abstracted data from included studies. Increasing awareness among physicians regarding the diagnosis of PID and the tendency of PID patients to develop cancer may lead to earlier diagnosis. Early diagnosis, appropriate treatment may decrease morbidity and mortality.
malignancy, primary immune deficiency, children, lymphoma
Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Pediatrics, Division of Pediatric Hematology-Oncology & Istanbul University, Oncology Institute, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Pediatrics, Division of Pediatric Allergy and Immunology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Pediatrics, Resident