Immunodeficiency

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Immunodeficiency due to a late component of complement deficiency

ORPHA:169150Disease

Also called Immunodeficiency due to C5 to C9 component complement deficiency · Terminal complement pathway deficiency

What it is

Immunodeficiency due to a late component of complement deficiency is a primary immunodeficiency due to an anomaly in either complement components C5, C6, C7, C8 or C9 and is typically characterized by meningitis due to often recurrent meningococcal infections. The prognosis is generally favorable.

Key facts

Inheritance
Autosomal recessive
Classified as
Disease

Genes

C5Disease-causing germline mutation(s)
C6Disease-causing germline mutation(s)
C7Disease-causing germline mutation(s)
C8ADisease-causing germline mutation(s)
C8BDisease-causing germline mutation(s)
C8GDisease-causing germline mutation(s)
C9Disease-causing germline mutation(s)

ICD-10 codes

D84.1filed under a broader ICD-10 category — shared with 15 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0015700OMIM 609536OMIM 610102OMIM 612446OMIM 613789OMIM 613790OMIM 613825UMLS C0398765

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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