Aim: To describe the demographic and clinical features of patients diagnosed with primary antibody deficiency
Material and Methods: The medical records of pediatric patients who were diagnosed with primary antibody deficiency were reviewed. Patients were diagnosed with primary antibody deficiency based on the European Society for Immunodeficiencies diagnostic criteria.
Result: A total of 60 patients with primary antibody deficiency were identified; 39 patients (65%) were male and median age was 5 (1-18) years of age, while median diagnosis age was 3 (6 mo-14 yr) years. Twenty-one patients (35%) were diagnosed with transient hypogammaglobulinemia of infancy, thirteen (21.7%) with selective Ig A deficiency, five (8.3%) with congenital agammaglobulinemia, five (8.3%) with selective Ig M deficiency, five (8.3%) with unclassified hypogammaglobulinemia, four (6.7%) with Ig G subclass deficiency, two (3.3%) with severe combined immune deficiency, two (3.3%) with common variable immunodeficiency, two (3.3%) with hyper Ig M syndrome, and one patient was diagnosed with pneumococcal vaccine responsiveness, respectively. Eleven patients (18.3%) had consanguinity and six patients (10%) had family history. The most common complaint was frequent respiratory tract infections. Four patients had dermatitis in addition to infection. Three patients had hematopoietic stem cell transplantation and 23 patients (38.3%) received intravenous immunoglobulin treatment. Two patients died during the follow up.
Conclusion: Our results indicated that diagnosis is delayed and the patients who are diagnosed with primary antibody deficiency frequently have respiratory tract infections.
Key words: Epidemiology; Intravenous Immunoglobulin; Primary Antibody Deficiency.
|
