Clinical and Developmental Immunology
Volume 2008 (2008), Article ID 624850, 10 pages
doi:10.1155/2008/624850
Review Article

Pediatric Selective IgM Immunodeficiency

Marc F. Goldstein,1 Alex L. Goldstein,2 Eliot H. Dunsky,1 Donald J. Dvorin,1 George A. Belecanech,1 and Kfir Shamir3

1The Asthma Center, Allergic Disease Associates, PC, 205 N. Broad street, Suite 300, Philadelphia, PA 19107, USA
2Wharton Undergraduate Division ('06), University of Pennsylvania, Philadelphia, PA 19104, USA
3Department of Medicine, Hahnemann University Hospital, Broad and Vine Streets, Philadelphia, PA 19102, USA

Received 8 February 2008; Revised 25 April 2008; Accepted 3 June 2008

Academic Editor: Chikao Morimoto

Copyright © 2008 Marc F. Goldstein et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective. Limited information exists on features of pediatric Selective IgM immunodeficiency (SIgMID). Previously published pediatric cases and 2 new cases are reviewed. Methods. English literature from PubMed and references from relevant articles were reviewed. Previously reported cases and 2 new cases from an allergy/immunology practice were analyzed. Results. Forty-nine reported cases of SIgMID presented with respiratory infections (77.6%), gastrointestinal disease (16.3%), skin disease (12.2%), and meningitis (8.2%). Mean serum IgM level was 16.5±13.8 mg/dL. Two patients were identified with SIgMID among 6300 active pediatric patients (0.03%) presenting with asthma, vasomotor rhinitis, and recurrent respiratory infections. In the 51 cases reported, none developed lymphoproliferative disease nor evolved into panhypogammaglobulinemia; four fatalities were reported. Conclusions. The prevalence of SIgMID in our pediatric population was 0.03%. In general, respiratory infections are the common comorbid conditions. Death and autoimmune disease are uncommon complications of pediatric SIgMID.