Humoral immune deficiencies

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X-linked agammaglobulinemia Btk defect (maturation arrest) ↓/absent B-cells
↓ Immunoglobulins
CVID Normal B-Cells
↓ Immunoglobulins
↑ risk of autoimmune disease, lymphoma
IgA deficiency (MCC cause) Normal B-cells
↓ IgA
↑ risk of autoimmune disease, anaphylaxis to IgA products
Hyper-IgM syndrome CD40L defect (no class switching) ↑ IgM
↓ IgA, IgG, IgE