Pediatric selective IgM immunodeficiency

Clin Dev Immunol. 2008:2008:624850. doi: 10.1155/2008/624850. Epub 2008 Nov 24.

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.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Autoantibodies / blood
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / immunology
  • Bacterial Infections / complications
  • Bacterial Infections / immunology
  • Chickenpox / complications
  • Chickenpox / immunology
  • Child
  • Child, Preschool
  • Dysgammaglobulinemia* / blood
  • Dysgammaglobulinemia* / complications
  • Dysgammaglobulinemia* / epidemiology
  • Dysgammaglobulinemia* / immunology
  • Dysgammaglobulinemia* / therapy
  • Female
  • Hemagglutinins / blood
  • Humans
  • Immunoglobulin M / deficiency*
  • Immunoglobulins, Intravenous / therapeutic use
  • Infant
  • Male
  • Mutation
  • PubMed
  • Respiratory Tract Infections / complications
  • Respiratory Tract Infections / immunology
  • Retrospective Studies

Substances

  • Autoantibodies
  • Hemagglutinins
  • Immunoglobulin M
  • Immunoglobulins, Intravenous