4A00.10

ICD-11 MMS 2026-01

Immunodeficiency with an early component of complement deficiency

Index terms

  • Immunodeficiency with an early component of complement deficiency
  • Deficiency of complement initial pathway
  • Complement component C1q deficiency
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  • Complement component C1r/C1s deficiency
  • Complement component C2 deficiency
  • Complement component C4 deficiency

Exclusions from above levels

  • 3A10.Y – Atypical haemolytic uraemic syndrome
  • 3A21.0 – Paroxysmal nocturnal haemoglobinuria
  • 02 – Neoplasms
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 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

Foundation entity

Immunodeficiency with an early component of complement deficiency – http://id.who.int/icd/entity/327609494

Part of: 4A00.1: Defects in the complement system › 4A00: Primary immunodeficiencies due to disorders of innate immunity › 04: Diseases of the immune system