Malignancy in common variable immunodeficiency: a systematic review and meta-analysis

Area of Science:

• Immunology
• Oncology

Background:

• Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disorder with diverse clinical features.
• CVID patients experience recurrent infections, autoimmune conditions, enteropathy, and increased risk of malignancy.
• Estimating the overall prevalence of malignancy in CVID patients is crucial for clinical management.

Purpose of the Study:

• To determine the overall prevalence of malignancy in patients diagnosed with Common Variable Immunodeficiency (CVID).
• To identify the specific types and prevalence of cancers occurring in CVID patients.
• To assess the association between malignancy and other CVID-related comorbidities like autoimmunity and malabsorption.

Main Methods:

• A systematic literature search was conducted across PubMed, Web of Science, and Scopus databases up to March 2019.
• Eligible studies involving Common Variable Immunodeficiency (CVID) patients were selected.
• A meta-analysis using random effects models was performed to pool malignancy prevalence estimates and confidence intervals.

Main Results:

• The meta-analysis included 48 studies encompassing 8123 CVID patients.
• The overall prevalence of malignancy in CVID patients was found to be 8.6% (95% CI: 7.1-10.0).
• Lymphoma (4.1%), gastric cancer (1.5%), and breast cancer (1.3%) were the most common malignancies. Autoimmunity and malabsorption were more prevalent in CVID patients with malignancy.

Conclusions:

• The prevalence of malignancy in Common Variable Immunodeficiency (CVID) patients has risen, potentially due to improved survival rates.
• Lymphoma is the predominant malignancy observed in this patient group.
• This study emphasizes the critical importance of regular malignancy screening and proactive management strategies for CVID patients.