This is a disease that occurs at any age. The peak incidence for this condition is the third decade.
The serum immunoglobulin levels in this condition are very variable. Usually there is no IgA, the serum IgG is below 200mg/100ml and the IgM is below 20mg/100ml.
In about 1/3 of patients there is severe lymphopaenia. In these patients there is often splenomegaly.
Recurrent pneumonia (leading to bronchiectasis), otitis media, sinusitis, and gastrointestinal infections are the most frequent infections. There is generally no predisposition to viral infections (if there is no lymphopaenia).
This condition is associated with autoimmune disorders.
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