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Original Article
 

Pediatric Patients With Common Variable Immunodeficiency: Long-term Follow-up
 

P Mohammadinejad,1 A Aghamohammadi,1 H Abolhassani,1 MS Sadaghiani,1 S Abdollahzade,1 B Sadeghi,1 H Soheili,1 M Tavassoli,1 SM Fathi,1 M Tavakol,1 N Behniafard,1 B Darabi,1 S Pourhamdi,1 N Rezaei1,2

1Research Center for Immunodefi ciencies, Pediatrics Center of Excellence, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
2Molecular Immunology Research, Center, and Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran

J Investig Allergol Clin Immunol 2012; Vol. 22(3): 208-214
 

 Abstract


Background: Common variable immunodeficiency (CVID) is the most common form of symptomatic primary immunodeficiency disease. It is characterized by hypogammaglobulinemia, increased predisposition to infections, autoimmunity, and cancer.

Objectives: This study was performed to evaluate the clinical and immunological features of a group of pediatric patients with CVID.

Methods: The study population comprised 69 individuals with CVID diagnosed during childhood.

Results: The patients were followed up for a mean (SD) period of 5.2 (4.3) years. The mean diagnostic delay was 4.4 (3.6) years, which was significantly lower in patients who were diagnosed recently. Children were classified according to 5 clinical phenotypes: infections only (n=39), polyclonal lymphocytic infiltration (n=17), autoimmunity (n=12), malignancy (n=7), and enteropathy (n=3). Postdiagnosis survival (10-year) was 71%.

Conclusions: The high percentages of pediatric patients with CVID in Iran may be due to the considerable prevalence of parental consanguinity in the region and an underlying genetic background.

Key words: Common variable immunodeficiency. Hypogammaglobulinemia. Pediatrics. Follow-up.