Intrinsic B-cell defect that prevents terminal maturation into Ab-secreting plasma cells.
Onset: adolescence, early adulthood
Spleen liver, lungs, skin: Noncaseating granuloma.
Increased incidence of pyogenic infection
Sprue-like GI syndrome
High risk of occurrence of B-cell neoplasms, gastric carcinoma, skin cancer.
DECREASED IgG
All Classes will be eventually affected.
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