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Evaluating laboratory criteria for combined immunodeficiency in adult patients diagnosed with common variable
Caroline von Spee-Mayer1, Verena Koemm2, Claudia Wehr3
1Department for Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany; Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.
Insights
Some patients diagnosed with common variable immunodeficiency (CVID) may actually have combined immunodeficiency (CID), requiring different treatments. Current diagnostic criteria are insufficient, though low naive CD4 T cells may indicate CID.
Area of Science:
- Immunology
- Clinical Medicine
- T-cell research
Background:
- Common variable immunodeficiency (CVID) is a heterogeneous group of primary immunodeficiencies.
- Some CVID patients may have underlying combined immunodeficiency (CID), necessitating distinct therapeutic strategies.
- Accurate differentiation between CVID and CID is crucial for appropriate patient management.
Purpose of the Study:
- To evaluate the efficacy of existing CD4 T-cell-based criteria in distinguishing CID from CVID in adult patients.
- To identify reliable immunological markers for diagnosing CID within the CVID cohort.
Main Methods:
- Retrospective analysis of immunological and clinical data from 238 adult CVID patients.
- Comparison of four proposed CD4 T-cell-based definitions for identifying CID.
- Assessment of marker sensitivity for clinical complications associated with CID.
Main Results:
- None of the evaluated criteria effectively separated CID from CVID patients.
- A relative reduction in naive CD4 T cells below 10% showed the highest sensitivity for identifying patients with CID-related clinical complications.
- Low naive CD4 T-cell counts warrant suspicion but are insufficient for a definitive CID diagnosis.
Conclusions:
- Current CD4 T-cell-based definitions are inadequate for differentiating CID from CVID in adult patients.
- While a low percentage of naive CD4 T cells is a potential indicator, it cannot solely define CID.
- Further research is needed to develop more precise diagnostic tools for combined immunodeficiency.
Abstract:
Some patients diagnosed with common variable immunodeficiency (CVID) actually suffer from combined immunodeficiency (CID) and therefore may require a different, CID-adapted treatment. Several CD4 T-cell-based criteria have been proposed in the past to identify patients with CID within the cohort of adult CVID patients. In this monocentric study, we used retrospective immunological and clinical data of 238 CVID patients to compare four different proposals of how to define CID among CVID patients. We demonstrate that none of the current definitions sufficiently separates CID from CVID patients and that the relative reduction of naïve CD4 T cells <10% has the highest sensitivity of all tested markers for patients with clinical complications often associated with CID. Thus, a very low percentage of naïve CD4 T cells in any adult CVID patient should raise suspicion, but is not sufficient to define CID among CVID patients.
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