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Diversity of malignancies in patients with different types of inborn errors of immunity.

ABSTRACT: Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 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. The frequency of malignancy and its association with the 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 (67.1%), followed by leukemia (11%), and cancers of the head and neck (7.3%). Among identified lymphoma cancers, non-Hodgkin's lymphomas were the most frequent type (43.9%) followed by different subtypes of Hodgkin's lymphoma (23.2%). Solid tumors (18.3%) appeared to be very heterogeneous by type and localization. The correlation between the type of malignancy and survival status and the association between the type of malignancy and IEI entities were unremarkable. The awareness of the association between the 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.

SUBMITTER: Tavakol M

PROVIDER: S-EPMC9743521 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

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Diversity of malignancies in patients with different types of inborn errors of immunity.

Tavakol Marzieh M Delavari Samaneh S Salami Fereshte F Ansari Sarina S Rasouli Seyed Erfan SE Chavoshzadeh Zahra Z Sherkat Roya R Ahanchian Hamid H Aleyasin Soheila S Esmaeilzadeh Hossein H Moazzen Nasrin N Shafiei Alireza A Abolnezhadian Farhad F Iranparast Sara S Ebrahimi Sareh Sadat SS Moeini Shad Tannaz T Pashangzadeh Salar S Nazari Farzad F Rezaei Arezou A Saeedi-Boroujeni Ali A Nabavi Mohammad M Arshi Saba S Fallahpour Morteza M Bemanian Mohammad Hassan MH Sharafian Samin S Shokri Sima S Eshaghi Sarvin S Nazari Shiva S Shamsian Bibi Shahin BS Dargahi Mal-Amir Mehrdad M Khazaei Roya R Ashkevari Pooya P Khavandegar Armin A Haghi Sabahat S Esmaeili Marzie M Abolhassani Hassan H Rezaei Nima N

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology 20221212 1

Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 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. The frequency of m ...[more]

PMID: 36510326

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Project description:Patients with inborn errors of immunity (IEI) in Argentina were encouraged to receive licensed Sputnik, AstraZeneca, Sinopharm, Moderna, and Pfizer vaccines, even though most of the data of humoral and cellular responses combination on available vaccines comes from trials conducted in healthy individuals. We aimed to evaluate the safety and immunogenicity of the different vaccines in IEI patients in Argentina. The study cohort included adults and pediatric IEI patients (n = 118) and age-matched healthy controls (HC) (n = 37). B cell response was evaluated by measuring IgG anti-spike/receptor binding domain (S/RBD) and anti-nucleocapsid(N) antibodies by ELISA. Neutralization antibodies were also assessed with an alpha-S protein-expressing pseudo-virus assay. The T cell response was analyzed by IFN-γ secretion on S- or N-stimulated PBMC by ELISPOT and the frequency of S-specific circulating T follicular-helper cells (TFH) was evaluated by flow cytometry.No moderate/severe vaccine-associated adverse events were observed. Anti-S/RBD titers showed significant differences in both pediatric and adult IEI patients versus the age-matched HC cohort (p < 0.05). Neutralizing antibodies were also significantly lower in the patient cohort than in age-matched HC (p < 0.01). Positive S-specific IFN-γ response was observed in 84.5% of IEI patients and 82.1% presented S-specific TFH cells. Moderna vaccines, which were mainly administered in the pediatric population, elicited a stronger humoral response in IEI patients, both in antibody titer and neutralization capacity, but the cellular immune response was similar between vaccine platforms. No difference in humoral response was observed between vaccinated patients with and without previous SARS-CoV-2 infection.In conclusion, COVID-19 vaccines showed safety in IEI patients and, although immunogenicity was lower than HC, they showed specific anti-S/RBD IgG, neutralizing antibody titers, and T cell-dependent cellular immunity with IFN-γ secreting cells. These findings may guide the recommendation for a vaccination with all the available vaccines in IEI patients to prevent COVID-19 disease.

Dataset Information

Diversity of malignancies in patients with different types of inborn errors of immunity.

Publications

Diversity of malignancies in patients with different types of inborn errors of immunity.

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