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• W4281293467 abstract "Abstract Genetic defects in development, maturation, and/or function of immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. Overall risk for cancer in children with IEI ranges from 4-25% and the type of malignancy is highly dependent on the specific mutant gene underlying IEI.We investigated 3056 IEI patients registered in the Iranian national registry between the years 1999 and 2020 in this retrospective cohort study. Frequency of malignancy and its association with type of IEI in these patients were evaluated.A total of 82 IEI patients with malignancy were enrolled in this study. Among them, predominantly lymphoma was the most common type of malignancy (65.8%), followed by leukemia (11%), and cancers of head and neck (7.3%). Among identified lymphoma cancers, Hodgkin’s lymphomas were the most frequent type (45.1%) followed by different subtypes of non-Hodgkin lymphoma (20.7%). Solid tumors (19.5%) appeared to be very heterogeneous by type and localization. The correlation between the type of malignancy and survival status and association between type of malignancy and IEI entities were unremarkable. The awareness of the association between presence of IEI and cancer highlights the importance of a synergistic effort by oncologists and immunologists in the early diagnosis of malignancy and personalized therapeutic strategies in IEI patients. Therefore, we investigated the frequency of different types of malignancy and also evaluated the correlation of type of IEI and type of malignancy in these 82 patients." @default.
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• W4281293467 date "2022-05-23" @default.
• W4281293467 modified "2023-10-15" @default.
• W4281293467 title "Diversity of Malignancies in Patients with Different Types of Inborn Errors of Immunity" @default.
• W4281293467 doi "https://doi.org/10.21203/rs.3.rs-1623394/v1" @default.
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