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Hypergammaglobulinemia in the pediatric population
© Lo and Sundel; licensee BioMed Central Ltd. 2012
Published: 13 July 2012
Hypergammaglobulinemia in adult patients is usually related to malignancy, autoimmune disease, or infection. The differential diagnosis of hypergammaglobulinemia in children has not been similarly well defined. Malignancies and autoimmunity are much less common in children compared to the adult population, and therefore the implications of an elevated immunoglobulin in a pediatric patient are less clear. We therefore sought to establish the differential diagnosis of hypergammaglobulinemia in children through a retrospective cohort analysis.
The Children’s Hospital Boston laboratory database was queried for all in- and out-patients with IgG levels ≥2000 mg/dL measured from 2000-2009. Charts were reviewed and patient characteristics, associated laboratory findings, diagnoses and outcomes were extracted. A random sampling of 10% of the patients was analyzed in detail.
Disease categories presenting with hypergammaglobulinemia
Among a cohort of patients at a large tertiary care children’s hospital with IgG level ≥2000 mg/dL, rheumatologic conditions constituted a much more frequent cause than infections. A wide variety of autoimmune diseases were represented, although not surprisingly lupus was the most frequent diagnosis. Interestingly, our cohort also included a large number of cystic fibrosis patients. The reason for hypergammaglobulinemia in many patients is not clear, though further studies may allow distinction between endogenous and exogenous lymphocyte stimulation.
Mindy S. Lo: None; Robert P. Sundel: None.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.