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

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Hereditary agammaglobulinaemia with profoundly reduced or absent B cells
• hereditary gamma globulin deficiency in blood
• hereditary agammaglobulinaemia antibody deficiency syndrome
• hereditary absence of gamma globulin in blood
• hereditary agammaglobulinaemia
• congenital sex-linked agammaglobulinaemia
• Isolated agammaglobulinaemia
• Autosomal recessive agammaglobulinaemia
• Agammaglobulinaemia, non-Bruton type
• Autosomal agammaglobulinaemia
• Swiss type autosomal recessive agammaglobulinaemia
• Swiss-type agammaglobulinaemia
• Ig beta deficiency
• Ig alpha deficiency
• Lambda 5 deficiency
• B-cell linker deficiency
• BLNK - [B-cell linker] deficiency
• X-linked agammaglobulinaemia
• Bruton agammaglobulinaemia
• Bruton type agammaglobulinaemia
• Bruton x-linked agammaglobulinaemia
• BTK - [Bruton tyrosine kinase] deficiency
• Bruton hypogammaglobulinaemia
• Syndromic agammaglobulinaemia
• Agammaglobulinaemia - microcephaly - craniosynostosis - severe dermatitis
• Immunodeficiency - centromeric region instability - facial anomalies syndrome
• Immunodeficiency - centromeric instability - facial anomalies
• Centromeric instability immunodeficiency syndrome
• Malignant myelodysplasia with hypogammaglobulinaemia
• Short stature due to growth hormone isolated deficiency with X-linked hypogammaglobulinaemia
• Hereditary hypogammaglobulinaemia antibody deficiency syndrome
• hereditary hypogammaglobulinaemia
• Severe combined immunodeficiency due to absent lymphoid stem cells
• SCID - [severe combined immunodeficiency] due to absent lymphoid stem cells

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