Clinical features and immunoglobulin replacement therapy outcomes of adults with common variable immunodeficiency: a single centre experience

Abstract

Background/aim: Common variable immunodeficiency (CVID) characterized by defective immunoglobulin production is the most prevalent form of symptomatic primary immunodeficiency (PID) in adults. We aimed to reveal the clinical features of adults with CVID and to evaluate the effects of immunoglobulin replacement treatment (IRT) on hemato-immunological findings.

Materials and methods: This study included 26 adult patients receiving IRT. Two measurements of complete blood counts and major immunoglobulin levels obtained at the beginning-end of follow up period were used for comparisons. Lymphocyte subsets and B-cell subgroups were measured only at the time of presentation.

Results: The most common complications were related to respiratory and digestive systems and organomegaly. Chronic diarrhoea and low body weight were positively correlated with the percentage of CD8(+) T cells (p = 0.019 and p = 0.003, respectively) but negatively correlated with the CD4/CD8 ratio and the percentage of CD19(+) B cells (p = 0.019 and p = 0.005 for both parameters, respectively). At the end of period, the distribution of haematological parameters significantly improved, and immunoglobulin M (IgM) level increased to detectable levels (p = 0.035).

Conclusions: There are apparent relationships among chronic diarrhoea and low body weight, and deterioration of T and B cell immunity in adults with CVID. IRT improves the whole blood parameters and stimulates immunoglobulin M (IgM) production. The later effect supports the immunomodulatory feature of this therapy.