4A01.00 category

Hereditary agammaglobulinaemia with profoundly reduced or absent B cells

Definition

This refers to a hereditary type of primary immune deficiency disease characterised by a reduction in all types of gamma globulins, and rare X-linked genetic disorder that affects the body's ability to fight infection.

Also indexed as

Agammaglobulinaemia - microcephaly - craniosynostosis - severe dermatitisAgammaglobulinaemia, non-Bruton typeAutosomal agammaglobulinaemiaAutosomal recessive agammaglobulinaemiaB-cell linker deficiencyBLNK - [B-cell linker] deficiencyBTK - [Bruton tyrosine kinase] deficiencyBruton agammaglobulinaemiaBruton hypogammaglobulinaemiaBruton type agammaglobulinaemiaBruton x-linked agammaglobulinaemiaCentromeric instability immunodeficiency syndromeHereditary agammaglobulinaemia with profoundly reduced or absent B cellsHereditary hypogammaglobulinaemia antibody deficiency syndromeIg alpha deficiencyIg beta deficiencyImmunodeficiency - centromeric instability - facial anomaliesImmunodeficiency - centromeric region instability - facial anomalies syndromeIsolated agammaglobulinaemiaLambda 5 deficiencyMalignant myelodysplasia with hypogammaglobulinaemiaSCID - [severe combined immunodeficiency] due to absent lymphoid stem cellsSevere combined immunodeficiency due to absent lymphoid stem cellsShort stature due to growth hormone isolated deficiency with X-linked hypogammaglobulinaemiaSwiss type autosomal recessive agammaglobulinaemiaSwiss-type agammaglobulinaemiaSyndromic agammaglobulinaemiaX-linked agammaglobulinaemiacongenital sex-linked agammaglobulinaemiahereditary absence of gamma globulin in bloodhereditary agammaglobulinaemiahereditary agammaglobulinaemia antibody deficiency syndromehereditary gamma globulin deficiency in bloodhereditary hypogammaglobulinaemia

Hereditary agammaglobulinaemia with profoundly reduced or absent B cells

Definition

Also indexed as

Nearby in Immunodeficiencies with predominantly antibody defects