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Inicio Allergologia et Immunopathologia Epidemiology and pathophysiology of malignancy in common variable immunodeficien...
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Vol. 45. Issue 6.
Pages 602-615 (November - December 2017)
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Vol. 45. Issue 6.
Pages 602-615 (November - December 2017)
DOI: 10.1016/j.aller.2017.01.006
Epidemiology and pathophysiology of malignancy in common variable immunodeficiency?
A. Tak Manesha, G. Azizib,c, A. Heydaric, F. Kiaeec, M. Shaghaghic, N. Hossein-Khannazerd, R. Yazdanie, H. Abolhassanic,f, A. Aghamohammadic,
Corresponding author

Corresponding author.
a Member of RACGP, Melbourne, Australia
b Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran
c Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
d Department of Immunology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran
e Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
f Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden
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Figures (1)
Tables (2)
Table 1. Frequency of different types of malignancy among different cohorts of CVID patients.
Table 2. The most common genetic mutations associated with malignancies in CVID patients.
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Common variable immunodeficiency (CVID) is a diagnostic category of primary immunodeficiency (PID) which may present with heterogeneous disorders including recurrent infections, autoimmunity, granulomatous diseases, lymphoid and other types of malignancies. Generally, the incidence of malignancy in CVID patients is around 1.5–20.7% and usually occurs during the 4th–6th decade of life. Non-Hodgkin lymphoma is the most frequent malignancy, followed by epithelial tumours of stomach, breast, bladder and cervix. The exact pathological mechanisms for cancer development in CVID are not fully determined; however, several mechanisms including impaired genetic stability, genetic predisposition, immune dysregulation, impaired clearance of oncogenic viruses and bacterial infections, and iatrogenic causes have been proposed to contribute to the high susceptibility of these patients to malignancies.

Common variable immunodeficiency


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