4A01.00
Hereditary agammaglobulinaemia with profoundly reduced or absent B cells
Agamaglobulinemia hereditária com células B profundamente reduzidas ou ausentes
CategoryDefinition
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.
Index Terms
Hereditary agammaglobulinaemia with profoundly reduced or absent B cellshereditary gamma globulin deficiency in bloodhereditary agammaglobulinaemia antibody deficiency syndromehereditary absence of gamma globulin in bloodhereditary agammaglobulinaemiacongenital sex-linked agammaglobulinaemiaIsolated agammaglobulinaemiaAutosomal recessive agammaglobulinaemiaAgammaglobulinaemia, non-Bruton typeAutosomal agammaglobulinaemiaSwiss type autosomal recessive agammaglobulinaemiaSwiss-type agammaglobulinaemiaIg beta deficiencyIg alpha deficiencyLambda 5 deficiencyB-cell linker deficiencyBLNK - [B-cell linker] deficiencyX-linked agammaglobulinaemiaBruton agammaglobulinaemiaBruton type agammaglobulinaemiaBruton x-linked agammaglobulinaemiaBTK - [Bruton tyrosine kinase] deficiencyBruton hypogammaglobulinaemiaSyndromic agammaglobulinaemiaAgammaglobulinaemia - microcephaly - craniosynostosis - severe dermatitisImmunodeficiency - centromeric region instability - facial anomalies syndromeImmunodeficiency - centromeric instability - facial anomaliesCentromeric instability immunodeficiency syndromeMalignant myelodysplasia with hypogammaglobulinaemiaShort stature due to growth hormone isolated deficiency with X-linked hypogammaglobulinaemiaHereditary hypogammaglobulinaemia antibody deficiency syndromehereditary hypogammaglobulinaemiaSevere combined immunodeficiency due to absent lymphoid stem cellsSCID - [severe combined immunodeficiency] due to absent lymphoid stem cells