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Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis
Saba Fekrvand1,2, Hassan Abolhassani1,3, Zahra Hamidi Esfahani1,2
1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Cancer is more common in patients with inborn errors of immunity (IEI) due to immune defects. This review highlights that cancer incidence in IEI patients depends on specific genetic defects, suggesting personalized screening and treatment can improve outcomes.
Area of Science:
- Immunology
- Oncology
- Genetics
Background:
- Patients with inborn errors of immunity (IEI) face a higher risk of developing cancer due to compromised immune systems.
- Cancer is a significant cause of mortality among individuals with IEI.
Approach:
- Systematic review and meta-analysis of demographic, genetic, and cancer data from 4607 IEI patients with malignancy history.
- Searched Embase, Web of Science, PubMed, and Scopus databases for studies published until September 2023.
Key Points:
- Hematologic/blood cancers, particularly diffuse large B-cell lymphoma, were the most frequent malignancies.
- ATM deficiency was most common among monogenic IEI defects, while NBS1 deficiency showed the highest cancer frequency rate.
- BRCA2 deficiency was associated with the earliest cancer onset, lowest cure rates, and highest fatality.
Conclusions:
- Overall cancer frequency in reviewed IEI cases was 11.1-12.0%.
- Cancer incidence in IEI is strongly linked to specific molecular and pathway defects.
- Individualized early screening and tailored treatments are crucial for improving prognosis in IEI patients.
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