Malignancies in children with immunodeficiencies

Abstract

Background. Primary immunodeficiency disorders (PIDs) are a heterogeneous group of genetic conditions characterized by impaired immune function. Recent studies have highlighted a complex connection between PIDs and an increased risk of malignancies. However, the underlying mechanisms remain poorly understood. Objective of the study. This study aims to understand the relationship between immunodeficiency and susceptibility to malignancy by reviewing literature sources. Material and methods. Data from online databases such as Pubmed, UpToDate, and the National Library of Medicine were used to review the literature examining factors associated with malignancy in patients with PID. Results. Cancer is the second most common cause of death in PID patients. The cancer risk in PID patients is estimated to be 4 - 25%, and some studies have reported a notable increase in standardized incidence ratios of various cancers. Non-Hodgkin lymphoma (48%) and Hodgkin lymphoma (10% of cases) are the most common cancers in PIDs, diagnosed often at younger ages. PID-related malignancies affect mainly ataxia-telangiectasia and common variable immunodeficiency patients over 50% of cases, another 30% linked to Wiskott-Aldrich syndrome, severe combined immunodeficiency and selective IgA deficiency. Conclusions. PID patients have a higher risk of many diverse cancers for genetic, immunological, and environmental reasons. Management is challenging, requiring continued surveillance and individualized treatment based on the underlying immunodeficiency and associated complications.