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The relationship between neutropenia and disease prognosis in patients with Common Variable Immunodeficiency (CVID)
Emrah Harman1, Fatih Çölkesen2, Mehmet Emin Gerek2
1Department of Internal Medicine, Division of Clinical Immunology and Allergy, Necmettin Erbakan University Faculty of Medicine, Konya, Türkiye; neueriskinimmunoloji@gmail.com.
Insights
Neutropenia is common in common variable immunodeficiency (CVID), affecting nearly 30% of patients. This condition is linked to significantly higher mortality rates and poorer survival in CVID patients.
Area of Science:
- Immunology
- Hematology
- Clinical Medicine
Background:
- Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired antibody production.
- Neutropenia, a low neutrophil count, can occur in CVID patients but its prognostic significance is not fully understood.
Purpose of the Study:
- To determine the frequency of neutropenia in CVID patients.
- To investigate the association between neutropenia and disease prognosis, including mortality and survival rates.
Main Methods:
- Retrospective review of data from 84 CVID patients (2019-2024).
- Patients categorized into neutropenic and non-neutropenic groups.
- Comparison of demographic, clinical, and laboratory parameters, including survival rates using Mann-Whitney U, Chi-square, and Kaplan-Meier analyses.
Main Results:
- Neutropenia was present in 28.5% of CVID patients.
- Neutropenic CVID patients exhibited significantly higher mortality rates (33.3% vs. 6.7%, P = 0.004).
- Kaplan-Meier analysis showed significantly lower 8-year survival rates for neutropenic CVID patients (57.5% vs. 92.5%, p < 0.001).
Conclusions:
- Neutropenia in CVID patients is associated with increased mortality.
- Neutropenia may serve as a critical clinical marker for disease prognosis in CVID.
- Close monitoring of neutropenia is crucial for managing CVID patients effectively.
Objective:
This study aimed to determine the frequency of neutropenia in patients with common variable immunodeficiency (CVID) and investigate its relationship with disease prognosis.
Methods:
Data from 84 patients diagnosed with CVID and followed between 2019 and 2024 at the Department of Adult Clinical Immunology and Allergy, Necmettin Erbakan University, were retrospectively reviewed. Patients were divided into two groups based on the presence or absence of neutropenia. Demographic data, clinical findings, laboratory parameters, and survival rates were compared. Statistical analyses included the Mann-Whitney U test, the Chi-square test, and the Kaplan-Meier survival analysis.
Results:
A total of 84 patients diagnosed with CVID were included in the study, with a median age of 38 years (range, 20-79 years). Of the participants, 48.8% were females (n = 41). Neutropenia was observed in 28.5% of patients (n = 24). The most common presenting complaints included autoimmune cytopenias, such as anemia and thrombocytopenia. Compared to non-neutropenic patients (n = 60), those with neutropenic CVID had a significantly higher mortality rate (33.3% vs 6.7%, P = 0.004). According to Kaplan-Meier survival analysis, the 8-year survival rates were 57.5 and 92.5% for neutropenic and for non-neutropenic CVID patients (p < 0.001), respectively.
Conclusion:
This study suggests that neutropenia in CVID patients may be more than just a hematological issue; it could also serve as an important clinical marker associated with increased mortality. Recognizing and closely monitoring neutropenia is essential for effective CVID management.
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