Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors

Clin Immunol. 2010 Dec;137(3):374-83. doi: 10.1016/j.clim.2010.08.010. Epub 2010 Sep 19.


We evaluated 131 children (M=88, F=43) with hypogammaglobulinemia. Data was analyzed mainly for delineating predictor factors for outcome. The distance from the lower limit of normal (-2SD) for any single measurement of immunoglobulins (Ig) was calculated and transformed into Ig scores. Mean age and duration of follow-up were 5.06 ± 4.05 and 3.7 ± 3.03 years, respectively. The diagnoses were: 22 CVID, 16 IgA deficiency, 33 transient hypogammaglobulinemia of childhood (THC), 3 selective IgM deficiency and 57 unclassified hypogammaglobulinemia (UCH). Low IgA scores (<-0.124) at presentation were indicative of subsequent development of IgA deficiency or CVID, whereas low IgM score (<-0.038) pointed towards more severe and persistent phenotypes. Combination of low IgM score between 2 and 5 years, impaired antibody response and low B cell counts enabled us to predict persistence of hypogammaglobulinemia beyond 5 years (specificity=90.5% and PPV=94.9%) and chronic lung disease (sensitivity=90.4% and specificity=68.3%). The set of criteria including low IgM scores, impaired antibody response and low B cell counts provided a high predictive value in detecting those with persistent hypogammaglobulinemia.

MeSH terms

  • Adolescent
  • Agammaglobulinemia / diagnosis*
  • Agammaglobulinemia / immunology*
  • Biomarkers
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Immunoglobulins / blood*
  • Male
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies


  • Biomarkers
  • Immunoglobulins