Llobet MP, Soler-Palacin P, Detkova D, Hernández M, Caragol I, Espanol T. Pediatr Allergy Immunol. 2009;20(2):113–118
PURPOSE OF THE STUDY. To describe and to classify children with common variable immunodeficiency (CVID) according to presentation, familial incidence, infections, and memory B (MB) cell classification.
STUDY POPULATION. Participants were children <18 years of age with CVID (N = 22) at a National Health Service referral center for immunodeficiency in Barcelona, Spain.
METHODS. A retrospective chart review was used to obtain clinical and immunologic data for pediatric patients with CVID monitored between 1985 and 2005. Clinical data included documentation of infections, onset of allergic diseases, autoimmune diseases, bronchiectasis, or cancer, and familial cases of CVID and other primary immunodeficiencies. Immunologic data included immunoglobulin levels, lymphocyte subsets, and classification of MB cells in patients with >2% CD19+ cells, to determine whether they were naive or mature. Patients with normal MB cells were classified as MB2, those with low MB cells but normal nonswitched MB cells were classified as MB1, and those with no MB cells were classified MB0.
RESULTS. The median age at diagnosis was 7.8 years (range: 2.5–16 years), with the exception of 1 outlier who was diagnosed at 6 months of age on the basis of family history and infectious manifestations. There were 15 boys and 7 girls, and follow-up periods ranged from 1 to 18 years. Infections were the most common manifestations before diagnosis, with respiratory and gastrointestinal symptoms being the most common. Infection rates improved with γ-globulin replacement therapy. Bronchiectasis secondary to respiratory infections was present in 7 of 22 patients and was the presenting finding for 5 of 7 patients. Allergy was diagnosed in 11 of 22 patients, but only 3 patients had positive specific allergen prick test results. CVID was diagnosed in 3 girls after they were found to have an autoimmune disease, which delayed the diagnosis for 1 patient who subsequently developed severe lung disease. Four patients had other family members with CVID or other primary immunodeficiency. Immunoglobulin G (IgG) levels (range: 80–552 mg/dL; median: 332 mg/dL) at the time of diagnosis did not correlate with the severity of clinical manifestations in children. Although results were not statistically significant, patients without MB cells (MB0 group) had more-severe complications, including bronchiectasis, persistent positive culture results, or autoimmune manifestations, compared with MB2 and MB1 groups.
CONCLUSIONS. Early diagnosis and treatment are important for patients with CVID. IgG levels at diagnosis did not correlate with the severity of clinical manifestations in pediatric patients with CVID; however, there was a trend suggesting that lack of MB cells might correlate with clinical severity and outcomes.
REVIEWERS COMMENTS. Contrary to findings among adult patients with CVID, IgG levels did not correlate with disease severity among children with CVID. Bronchiectasis was diagnosed in one third of pediatric patients with CVID and presented as early as 2.5 years of age. Providers should consider CVID in the differential diagnosis for young children with recurrent infections. The authors were able to demonstrate that MB cell classification correlates with the severity of clinical manifestations and may be an important marker to aid in the determination of prognoses for patients with CVID in the future.
- Copyright © 2009 by the American Academy of Pediatrics