4A00.1

ICD-11 MMS 2026-01

Defects in the complement system

Excludes

  • 3A10.Y – Atypical haemolytic uraemic syndrome
  • 3A21.0 – Paroxysmal nocturnal haemoglobinuria

Exclusions from above levels

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

Child codes

  • 4A00.10 – Immunodeficiency with an early component of complement deficiency
  • 4A00.11 – Immunodeficiency with a late component of complement deficiency
  • 4A00.12 – Immunodeficiency with factor B deficiency
  • 4A00.13 – Immunodeficiency with factor D anomaly
  • 4A00.14 – Hereditary angioedema
  • 4A00.15 – Acquired angioedema
  • 4A00.1Y – Other specified defects in the complement system
  • 4A00.1Z – Defects in the complement system, unspecified

Sibling codes

  • 4A00.0 – Functional neutrophil defects
  • 4A00.2 – Genetic susceptibility to particular pathogens
  • 4A00.3 – Immunodeficiency with natural-killer cell deficiency
  • 4A00.Y – Other specified primary immunodeficiencies due to disorders of innate immunity
  • 4A00.Z – Primary immunodeficiencies due to disorders of innate immunity, unspecified

Foundation entity

Defects in the complement system – http://id.who.int/icd/entity/1222145690

Part of: 4A00: Primary immunodeficiencies due to disorders of innate immunity › 04: Diseases of the immune system