4A01.00

ICD-11 MMS 2026-01

Hereditary agammaglobulinaemia with profoundly reduced or absent B cells

Description

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 cells
  • congenital sex-linked agammaglobulinaemia
  • hereditary absence of gamma globulin in blood
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  • hereditary agammaglobulinaemia
  • hereditary agammaglobulinaemia antibody deficiency syndrome
  • hereditary gamma globulin deficiency in blood
  • 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
  • BTK - [Bruton tyrosine kinase] deficiency
  • Bruton agammaglobulinaemia
  • Bruton hypogammaglobulinaemia
  • Bruton type agammaglobulinaemia
  • Bruton x-linked agammaglobulinaemia
  • Syndromic agammaglobulinaemia
  • Agammaglobulinaemia - microcephaly - craniosynostosis - severe dermatitis
  • Immunodeficiency - centromeric region instability - facial anomalies syndrome
  • Centromeric instability immunodeficiency syndrome
  • Immunodeficiency - centromeric instability - facial anomalies
  • 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

Exclusions from above levels

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A01.01 – Immunodeficiencies with severe reduction in at least two serum immunoglobulin isotypes with normal or low numbers of B cells
  • 4A01.02 – Specific antibody deficiency with normal immunoglobulin concentrations or normal number of B cells
  • 4A01.03 – Transient hypogammaglobulinaemia of infancy
  • 4A01.04 – Immunodeficiencies with isotype or light chain deficiencies with normal number of B cells
  • 4A01.05 – Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells
  • 4A01.0Y – Other specified immunodeficiencies with predominantly antibody defects
  • 4A01.0Z – Immunodeficiencies with predominantly antibody defects, unspecified

Foundation entity

Hereditary agammaglobulinaemia with profoundly reduced or absent B cells – http://id.who.int/icd/entity/393046642

Part of: 4A01.0: Immunodeficiencies with predominantly antibody defects › 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system