Table 3.

Single gene defects of the immune system resulting in antibody deficiencies.

Agammaglobulinemia with absent B cells 
X-linked agammaglobulinemia (Bruton’s) 85% 
μ heavy chain 5% 
Surrogate light chain < 1% 
Igα/Igβ 2% 
BLNK 2% 
Hyper IgM syndrome 
CD40 ligand 65% 
CD40 < 5% 
AID 15% 
NEMO 10% 
Agammaglobulinemia with absent B cells 
X-linked agammaglobulinemia (Bruton’s) 85% 
μ heavy chain 5% 
Surrogate light chain < 1% 
Igα/Igβ 2% 
BLNK 2% 
Hyper IgM syndrome 
CD40 ligand 65% 
CD40 < 5% 
AID 15% 
NEMO 10% 
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