Table III.

Primary immune deficiencies occurring with agammaglobulinemia or hypogammaglobulinemia according to (ESID) [20]

Agammaglobulinemia

Bloom syndrome

CSR defects (defects of class-switch recombination and HIGM syndromes)

Common variable immunodeficiency disorders (CVID)

HLA class II deficiency (MHC2)

IgA with IgG subclass deficiency

Immunodeficiency centromeric instability facial anomalies syndrome (ICF)

Isolated IgG subclass deficiency

Nijmegen breakage syndrome

Selective IgA deficiency

Selective IgM deficiency

Thymoma with immunodeficiency

Transient hypogammaglobulinaemia of infancy

Wart, hypogammaglobulinemia infections and myelokathexis (WHIM)

X-linked lymphoproliferative syndrome (XLP)

Unclassified antibody deficiency