4A01.0Level 4

Immunodeficiencies with predominantly antibody defects

**Definition:** A disorder characterised by an inability to mount a normal immune response due to antibody (i.e. immunoglobulin) defects

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4A01.00Hereditary agammaglobulinaemia with profoundly reduced or absent B cells

4A01.01Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells

4A01.02Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells

4A01.03Transient hypogammaglobulinaemia of infancy

4A01.04Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells

4A01.05Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells

4A01.0YOther specified immunodeficiencies with predominantly antibody defects

4A01.0ZImmunodeficiencies with predominantly antibody defects, unspecified

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