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4A00.1 Defects in the complement system

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

exclusions

• Atypical haemolytic uraemic syndrome  (3A10)
• Paroxysmal nocturnal haemoglobinuria  (3A21.0)

sections/codes in this section (4A00.1-4A00.1)

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

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