The evaluation of neutropenia in common variable immune deficiency patients

ABSTRACT

Objectives: Common variable immunodeficiency is a primary immunodeficiency disease characterized by hypogammaglobulinemia and heterogeneous clinical features. Neutropenia is a rare complication among CVID patients leading to a higher rate of infections and morbidity. Multiple factors (e.g. autoimmunity, infections, drugs and etc.) are found to underlie this complication.

Methods: In the present study, demographic, clinical and laboratory data were compared between two groups of CVID patients with and without neutropenia.

Results: Frequency of neutropenia was 8.1%. Infectious complications were the most prevalent clinical manifestations regardless of presence of neutropenia. However, candida infection and septicemia were significantly higher in neutropenic patients (p = 0.001 and p = 0.01, respectively). The most prominent clinical phenotypes of CVID patients with neutropenia were polyclonal lymphocytic infiltration and autoimmunity, both being considerably higher compared to the non-neutropenic group (p = 0.04 and p = 0.009, respectively). The mortality rate in neutropenic patients was higher than in patients without neutropenia (61.1 vs. 25.2%, p = 0.004).

Conclusion: Although neutropenia is a rare complication among CVID patients, it is associated with frequent and severe clinical complications, including autoimmunity and lymphoproliferative conditions. Also, its accompaniment with higher mortality frequency in CVID patients indicates a need for more precise attention and consideration regarding specific treatment in neutropenic patients.

KEYWORDS:

• Primary immunodeficiency diseases
• common variable immunodeficiency
• neutropenia
• autoimmune cytopenia
• hypersplenism

Article highlights

• The frequency of neutropenia was 8.1% among Iranian CVID patients.

• Infectious complications were the most common clinical manifestations regardless of the presence of neutropenia.

• The polyclonal lymphocytic infiltration and autoimmunity were the prominent non-infectious clinical features in CVID patients with neutropenia.

• The mortality rate was higher in neutropenic patients than those without it.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This work was supported by the vice chancellor for research, Tehran University of Medical Sciences, under Grant No. 35731.