ICD-11 code : 4A01.00;
Preferred Label : Hereditary agammaglobulinaemia with profoundly reduced or absent B cells;
ICD-11 definition : This refers to a hereditary type of primary immune deficiency disease characterized
by a reduction in all types of gamma globulins, and rare X-linked genetic disorder
that affects the body's ability to fight infection.;
ICD-11 synonym : hereditary agammaglobulinaemia; hereditary gamma globulin deficiency in blood; hereditary hypogammaglobulinaemia; congenital sex-linked agammaglobulinaemia; hereditary agammaglobulinaemia antibody deficiency syndrome; hereditary absence of gamma globulin in blood;
ICD-11 inclusion : SCID - [severe combined immunodeficiency] due to absent lymphoid stem cells; hereditary hypogammaglobulinaemia antibody deficiency syndrome; Swiss-type agammaglobulinaemia; Swiss type autosomal recessive agammaglobulinaemia;
Origin ID : 393046642;
ICD-10 Mapping
This refers to a hereditary type of primary immune deficiency disease characterized
by a reduction in all types of gamma globulins, and rare X-linked genetic disorder
that affects the body's ability to fight infection.
