Common Variable Immunodeficiency-A Case Report with Emphasis on the Diagnostic Clues and Treatment Response

Abstract

Common variable immunodeficiency (CVID) encompasses a group of heterogeneous conditions linked by a lack of immunoglobulin production and primary antibody failure. CVID has a broad range of clinical symptoms with the involvement of multiple organs, especially the respiratory system. The mean onset of symptoms in patients with CVID is in their 3rd decade of life. Given the complexity and rarity of the disease, the diagnosis of CVID is often delayed for a mean of 8.9 years. We herein report a 24-year-old female patient with CVID manifesting with migratory pneumonia, splenomegaly, and persistently elevated liver enzymes. The disease was suspected due to the presence of hypoglobulinemia, and confirmed by low serum levels of IgG, IgM, and IgA. Her splenomegaly was attributed to CVID-related lymphoproliferative disorder. The spleen regressed to normal size after intravenous immunoglobulin supplementation. This case emphasizes the need for a high index of clinical suspicion for CVID in patients presenting with recurrent sinopulmonary infections and/or an impaired capacity to produce specific antibodies in response to infection, such as chronic hepatitis C. Early diagnosis is needed to prevent significant morbidity and mortality and to improve the prognosis in these patients.