Primary immunodeficiencies: Clinical spectrum and follow-up challenges

Primary immunodeficiencies (PIDs) are a rare group of diseases with a broad clinical spectrum. Patients usually present with infections; they may also present with autoimmunity, lymphoproliferative disorders, and/or cancers or these comorbidities may occur during the disease. We aimed to analyze the demographic and clinical characteristics of adult patients with PID and evaluate the relationship between laboratory values and comorbidities with disease and each other. This study included 55 adult PID patients receiving immunoglobulin replacement therapy. Demographic characteristics, laboratory parameters, treatment modalities, and clinical course of patients were recorded. The most common PID subtype was Common Variable Immunodeficiency. The most common initial complaints were related to the respiratory and digestive systems; 11 cancers were detected in 9 patients. None of the immunodeficiency patients with cancer were in the underweight group. IgA and platelet levels were higher in the group that developed cancer, and four patients have deceased. PIDs are multisystemic diseases. Early diagnosis and prompt treatment can lead to significant improvements in morbidity and mortality. Increased awareness and interdisciplinary cooperation are important.