Terminal complement pathway deficiency
| Terminal complement pathway deficiency |
|---|
Terminal complement pathway deficiency is a genetic condition affecting the complement membrane attack complex (MAC).
It involves deficiencies of C5, C6, C7, and C8. (While C9 is part of the MAC, and deficiencies have been identified,[1] it is not required for cell lysis.[2])
People with this condition are prone to meningococcal infection.[3] Vaccination may be recommended.[4]
Diagnosis
| C4 (C) | FB (A) | C3 | CH50 | Conditions |
|---|---|---|---|---|
| · | ↓ | ↓ | ↓ | PSG, C3 NeF AA |
| ↓ | · | ↓ | · | HAE, C4D |
| · | · | · | ↓ | TCPD |
| ↓ | ·/↓ | ↓ | ↓ | SLE |
| ↑ | ↑ | ↑ | ↑ | inflammation |
Initial complement tests often include C3 and C4, but not C5 through C9. Instead, the CD50 result may play a role in diagnosis: if the CD50 level is low but CD3 and CD4 are normal, then analysis of the individual terminal components may be warranted.
References
- ^ Lint TF, Zeitz HJ, Gewurz H (1980). "Inherited deficiency of the ninth component of complement in man". J. Immunol. 125 (5): 2252–7. PMID 7430628.
{{cite journal}}: Unknown parameter|month=ignored (help)CS1 maint: multiple names: authors list (link) - ^ Thomas M. Habermann; Mayo Clinic (1 November 2007). Mayo Clinic Internal Medicine Concise Textbook. CRC Press. pp. 30–. ISBN 9781420067491. Retrieved 14 November 2010.
- ^ J.K. Sinha & S. Bhattacharya. A Text Book of Immunology. Academic Publishers. pp. 385–. ISBN 9788189781095. Retrieved 14 November 2010.
- ^ Frederick S. Southwick (10 December 2007). Infectious diseases: a clinical short course. McGraw Hill Professional. pp. 149–. ISBN 9780071477222. Retrieved 14 November 2010.
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