Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity
Inborn errors of immunity (IEI) are a heterogeneous group of inherited disorders, and almost 500 genes associated with these disorders have been identified. Defects in IEI genes lead to diverse clinical manifestations including increased susceptibility to recurrent or prolonged infections, immune dy...
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-01-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/15/3/764 |
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| author | Samaneh Delavari Yating Wang Tannaz Moeini shad Salar Pashangzadeh Farzad Nazari Fereshte Salami Hassan Abolhassani |
| author_facet | Samaneh Delavari Yating Wang Tannaz Moeini shad Salar Pashangzadeh Farzad Nazari Fereshte Salami Hassan Abolhassani |
| author_sort | Samaneh Delavari |
| collection | DOAJ |
| description | Inborn errors of immunity (IEI) are a heterogeneous group of inherited disorders, and almost 500 genes associated with these disorders have been identified. Defects in IEI genes lead to diverse clinical manifestations including increased susceptibility to recurrent or prolonged infections, immune dysregulation phenotypes (such as severe atopy, allergy, autoimmunity, and uncontrolled inflammation, lymphoproliferation), as well as predisposition to malignancies. Although the majority of IEI patients present hematologic cancers, the characteristics of other types of cancers are not well described in these groups of patients. By investigating 5384 IEI patients registered in the Iranian national registry the clinical and immunologic phenotypes of patients with non-hematologic cancers were compared with other malignant and non-malignant patients. Solid tumors were reported <20% of malignant IEI patients (<i>n</i> = 27/144 patients) and appeared to be very heterogeneous by type and localization as well as molecular defects (mainly due to DNA repair defect resulted from ATM deficiency). The correlation between the type of malignancy and survival status was remarkable as patients with non-hematologic cancers survive higher than IEI patients with hematologic cancers. Our findings showed that different types of malignancy could be associated with specific entities of IEI. Therefore, the education of physicians about the risk of malignancies in IEI is required for personalized treatment and appropriate management of patients. |
| first_indexed | 2024-03-11T09:50:13Z |
| format | Article |
| id | doaj.art-8e169c95097345a9890457a11f20b901 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-11T09:50:13Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-8e169c95097345a9890457a11f20b9012023-11-16T16:16:51ZengMDPI AGCancers2072-66942023-01-0115376410.3390/cancers15030764Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of ImmunitySamaneh Delavari0Yating Wang1Tannaz Moeini shad2Salar Pashangzadeh3Farzad Nazari4Fereshte Salami5Hassan Abolhassani6Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranDivision of Clinical Immunology, Department of Biosciences and Nutrition, Karolinska Institute, 14183 Stockholm, SwedenResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranResearch Center for Immunodeficiencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Science, 1419733151 Tehran, IranInborn errors of immunity (IEI) are a heterogeneous group of inherited disorders, and almost 500 genes associated with these disorders have been identified. Defects in IEI genes lead to diverse clinical manifestations including increased susceptibility to recurrent or prolonged infections, immune dysregulation phenotypes (such as severe atopy, allergy, autoimmunity, and uncontrolled inflammation, lymphoproliferation), as well as predisposition to malignancies. Although the majority of IEI patients present hematologic cancers, the characteristics of other types of cancers are not well described in these groups of patients. By investigating 5384 IEI patients registered in the Iranian national registry the clinical and immunologic phenotypes of patients with non-hematologic cancers were compared with other malignant and non-malignant patients. Solid tumors were reported <20% of malignant IEI patients (<i>n</i> = 27/144 patients) and appeared to be very heterogeneous by type and localization as well as molecular defects (mainly due to DNA repair defect resulted from ATM deficiency). The correlation between the type of malignancy and survival status was remarkable as patients with non-hematologic cancers survive higher than IEI patients with hematologic cancers. Our findings showed that different types of malignancy could be associated with specific entities of IEI. Therefore, the education of physicians about the risk of malignancies in IEI is required for personalized treatment and appropriate management of patients.https://www.mdpi.com/2072-6694/15/3/764inborn errors of immunityprimary immunodeficiencymalignancysolid tumors |
| spellingShingle | Samaneh Delavari Yating Wang Tannaz Moeini shad Salar Pashangzadeh Farzad Nazari Fereshte Salami Hassan Abolhassani Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity Cancers inborn errors of immunity primary immunodeficiency malignancy solid tumors |
| title | Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity |
| title_full | Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity |
| title_fullStr | Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity |
| title_full_unstemmed | Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity |
| title_short | Clinical and Immunologic Characteristics of Non-Hematologic Cancers in Patients with Inborn Errors of Immunity |
| title_sort | clinical and immunologic characteristics of non hematologic cancers in patients with inborn errors of immunity |
| topic | inborn errors of immunity primary immunodeficiency malignancy solid tumors |
| url | https://www.mdpi.com/2072-6694/15/3/764 |
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