Fatemeh Kiaee1,2, Gholamreza Azizi3, Hosein Rafiemanesh4, Hamed Zainaldain2, Fatema Sadaat Rizvi2, Mahla Alizadeh5,6, Mahnaz Jamee6, Sara Mohammadi2, Sima Habibi2, Laleh Sharifi7, Farhad Jadidi-Niaragh8,9, Sabahat Haghi10, Reza Yazdani2, Hassan Abolhassani2,11, Asghar Aghamohammadi2. 1. Department of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences , Tehran , Iran. 2. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences , Tehran , Iran. 3. Non-communicable Diseases Research Center, Alborz University of Medical Sciences , Karaj , Iran. 4. Student Research Committee, Department of Epidemiology, School of Public Health, Shahid Beheshti University of Medical Sciences , Tehran , Iran. 5. Evidence- Based Phytotherapy and Complementary Medicine Research Center, Alborz University of Medical Sciences , Karaj , Iran. 6. Student Research Committee, Alborz University of Medical Sciences , Karaj , Iran. 7. Uro-Oncology Research Center, Tehran University of Medical Sciences , Tehran , Iran. 8. Immunology Research Center, Tabriz University of Medical Sciences , Tabriz , Iran. 9. Department of Immunology, School of Medicine, Tabriz University of Medical Sciences , Tabriz , Iran. 10. Department of Hematology & Oncology, School of Medicine, Alborz University of Medical Sciences , Karaj , Iran. 11. Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge , Stockholm , Sweden.
Abstract
Background: Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency (PID) disorder characterized by variable clinical manifestations including recurrent infections, autoimmune disorders, enteropathy, lymphoproliferative disorders, and malignancy. The aim of this study is to estimate the overall prevalence of malignancy in patients with CVID. Methods: PubMed, Web of Science and Scopus were searched systemically to find eligible studies from the earliest available date to March 2019 with standard keywords. Pooled estimates of the malignancy prevalence and the corresponding 95% confidence intervals (CI) were calculated using random effects models. Results: Forty-eight studies with a total of 8123 CVID patients met the inclusion criteria and were finally included in the meta-analysis. Overall prevalence of malignancy was 8.6% (95% CI: 7.1-10.0; I2 = 79.2%). The prevalence of lymphoma, gastric cancer, and breast cancer in CVID patients were 4.1% (95% CI: 3.3-4.9; I2 = 62.6%), 1.5% (95% CI: 0.78-2.2; I2 = 68.9%), and 1.3% (95% CI: 0.64-1.9; I2 = 54.9%), respectively. Moreover, autoimmunity and malabsorption were more frequent in patients with malignancy than those without malignancy. Conclusion: The prevalence of malignancy has increased in CVID patients due to recent improvement in survival rate and the lymphoma is the most common type. This research highlighted the significance of malignancy screening and management in CVID patients.
Background: Common variable immunodeficiency (CVID) is the most common clinically significant primary immunodeficiency (PID) disorder characterized by variable clinical manifestations including recurrent infections, autoimmune disorders, enteropathy, lymphoproliferative disorders, and malignancy. The aim of this study is to estimate the overall prevalence of malignancy in patients with CVID. Methods: PubMed, Web of Science and Scopus were searched systemically to find eligible studies from the earliest available date to March 2019 with standard keywords. Pooled estimates of the malignancy prevalence and the corresponding 95% confidence intervals (CI) were calculated using random effects models. Results: Forty-eight studies with a total of 8123 CVIDpatients met the inclusion criteria and were finally included in the meta-analysis. Overall prevalence of malignancy was 8.6% (95% CI: 7.1-10.0; I2 = 79.2%). The prevalence of lymphoma, gastric cancer, and breast cancer in CVIDpatients were 4.1% (95% CI: 3.3-4.9; I2 = 62.6%), 1.5% (95% CI: 0.78-2.2; I2 = 68.9%), and 1.3% (95% CI: 0.64-1.9; I2 = 54.9%), respectively. Moreover, autoimmunity and malabsorption were more frequent in patients with malignancy than those without malignancy. Conclusion: The prevalence of malignancy has increased in CVIDpatients due to recent improvement in survival rate and the lymphoma is the most common type. This research highlighted the significance of malignancy screening and management in CVIDpatients.
Entities:
Keywords:
Malignancy; common variable immunodeficiency; lymphoma; primary immunodeficiency
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