Table II.

Causes of primary antibody deficiency in our cohort of patients

Cause of PAD	No. of patients
CVID	101
Selective antibody deficiency	12
Possible CVID	6
IgG subclass deficiency	5
X-linked syndromes	5
Good syndrome	4
Panhypogammaglobulinemia with no secondary cause	4
Hyper-IgM syndrome	2
Others∗	8

CVID, Common variable immune deficiency.

^∗

Others consists of 1 case each of the following:

• •
  Combined immune deficiency—hemizygous for c.98T>A, p.(Ile33Asn) in CD40LG likely causal variant for X-linked immunodeficiency.
• •
  Autoimmune polyendocrinopathy syndrome type 1 due to a mutation in the autoimmune regulator gene with no detectable CD19⁺ B cells.
• •
  Primary immunodeficiency, combined T- and B-cell deficiency, mimicking, but not due to CTLA4 deficiency.
• •
  Roifman syndrome.
• •
  Hyper-IgE syndrome.
• •
  PAD, common gamma-chain (γc) mutation associated with significant cell-mediated immunodeficiency and absent natural killer cells.
• •
  Hypogammaglobulinemia with DOCK8 mutation.
• •
  Dysgammaglobulinemia with impaired T-cell receptor Vβ repertoire.