4A01.2

ICD-11 MMS 2026-01

Diseases of immune dysregulation

Exclusions from above levels

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

Child codes

  • 4A01.20 – Immune dysregulation syndromes with hypopigmentation
  • 4A01.21 – Immune dysregulation syndromes presenting primarily with autoimmunity
  • 4A01.22 – Immune dysregulation syndromes presenting primarily with lymphoproliferation
  • 4A01.23 – Primary haemophagocytic lymphohistiocytosis
  • 4A01.2Y – Other specified diseases of immune dysregulation
  • 4A01.2Z – Diseases of immune dysregulation, unspecified

Sibling codes

  • 4A01.0 – Immunodeficiencies with predominantly antibody defects
  • 4A01.1 – Combined immunodeficiencies
  • 4A01.3 – Other well-defined immunodeficiency syndromes due to defects in adaptive immunity
  • 4A01.Z – Primary immunodeficiencies due to disorders of adaptive immunity, unspecified

Foundation entity

Diseases of immune dysregulation – http://id.who.int/icd/entity/1674805091

Part of: 4A01: Primary immunodeficiencies due to disorders of adaptive immunity › 04: Diseases of the immune system