- This is acquired agammaglobulinemia (second and third decade of life).
- Males and females are equally affected.
- Cause is unknown, but viral infection (Epstain-Barr) has been implicated.
- B-cells are present but do not produce IgG-s.
- Activation of B-cells by the T-cells is defective.
- Patients are prone to pyogenic infections and to gastrointestinal protozoan Giardia lamblia (which causes severe diarrhea).
- Many patients develop autoimmune disease (pernicious anemia) for unknown reasons.
- Splenomegaly and generalized lymphadenopathy are present in many patients with common variable immunodeficiency (CVID).
Malignancy
- In patients with CVID, the risk of certain malignancies is high.
- Lymphomas of a B-cell phenotype are of particular concern.
- Malignancy is most likely associated with the Epstein-Barr virus.
- The risk of gastric carcinoma is almost 50 times greater in patients with CVID than in other individuals.
- Malignant melanomas are reported.
