4A01.05

ICD-11 MMS 2026-01

Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells

Index terms

  • Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells
  • Hyper IgM
  • Activation-induced cytidine deaminase deficiency
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  • HIGM2 - [hyper-IgM syndrome type 2]
  • Hyper-IgM syndrome due to AICDA deficiency
  • Hyper-IgM syndrome due to AID deficiency
  • Hyper-IgM syndrome type 2
  • Hyper-IgM syndrome due to uracil N glycosylase
  • HIGM5 - [hyper-IgM syndrome type 5]
  • Hyper-IgM syndrome due to UNG - [uracil N glycosylase] deficiency
  • Hyper-IgM syndrome type 5

Exclusions from above levels

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

Sibling codes

  • 4A01.00 – Hereditary agammaglobulinaemia with profoundly reduced or absent B cells
  • 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
  • 4A01.0Y – Other specified immunodeficiencies with predominantly antibody defects
  • 4A01.0Z – Immunodeficiencies with predominantly antibody defects, unspecified

Foundation entity

Immunodeficiencies with severe reduction in serum IgG or IgA with normal or elevated IgM and normal numbers of B-cells – http://id.who.int/icd/entity/769068598

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