Abstract
Of all primary immunodeficiencies, predominantly antibody deficiencies (PADs) form the largest group. Within this group, clinical severity as well as the results of immunological laboratory investigations and potential underlying pathophysiology may differ greatly. Milder forms of PAD are often grouped together as hypogammaglobulinemia; this can be found at any age; however, the clinical significance may differ in different age groups. In some children, immunoglobulin production matures more slowly, resulting in so-called transient hypogammaglobulinemia of infancy. Milder hypogammaglobulinemia patients are often missed, because of lack of awareness and—even when immunologically screened—incomplete investigations. Patients generally present with recurrent “normal” ENT and airway infections. Chronic fatigue is seen in many patients, leading to decreased life quality and less societal participation. In general, combinations of abnormal laboratory results seem to be associated with more symptoms and more sequelae. It is currently unknown how the different forms of milder hypogammaglobulinemia arise.
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de Vries, E. (2019). IgG Subclass and Anti-polysaccharide Antibody Deficiency. In: D'Elios, M., Rizzi, M. (eds) Humoral Primary Immunodeficiencies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-91785-6_17
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