Skip to main content
NCBI home page
British Medical Journal logoLink to British Medical Journal
. 1976 Feb 14;1(6006):367–369. doi: 10.1136/bmj.1.6006.367

Deficiency of factor B of the complement system in sickle cell anaemia.

W A Wilson, G R Hughes, P J Lachmann
PMCID: PMC1638784  PMID: 1247858

Abstract

Factors B and D as well as the total activity of the alternative pathway of complement activation were measured using a functional assay in sera from 29 patients with sickle cell anaemia and 18 normal controls. Total alternative pathway activity was reduced in the patients compared with controls. In patients with abnormally low total alternative pathway activity factor D levels were normal, whereas factor B levels were significantly depressed to a mean level of about half of normal. Regression analysis in patients also showed a significant relation between total alternative pathway activity and factor B levels. A deficiency of factor B is the likely cause of the defect in the complement system in patients with sickle cell anaemia. Such a defect may contribute to the excessive proneness of such patients to severe infection.

Full text

PDF
367

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Eeckels R., Gatti F., Renoirte A. M. Abnormal distribution of haemoglobin genotypes in Negro children with severe bacterial infections. Nature. 1967 Oct 28;216(5113):382–382. doi: 10.1038/216382a0. [DOI] [PubMed] [Google Scholar]
  2. Fearon D. T., Austen K. F., Ruddy S. Formation of a hemolytically active cellular intermediate by the interaction between properdin factors B and D and the activated third component of complement. J Exp Med. 1973 Dec 1;138(6):1305–1313. doi: 10.1084/jem.138.6.1305. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Fearon D. T., Austen K. F., Ruddy S. Properdin factor D: characterization of its active site and isolation of the precursor form. J Exp Med. 1974 Feb 1;139(2):355–366. doi: 10.1084/jem.139.2.355. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Goodkofsky I., Lepow I. H. Functional relationship of factor B in the properdin system to C3 proactivator of human serum. J Immunol. 1971 Oct;107(4):1200–1204. [PubMed] [Google Scholar]
  5. Johnston R. B., Jr, Newman S. L., Struth A. G. An abnormality of the alternate pathway of complement activation in sickle-cell disease. N Engl J Med. 1973 Apr 19;288(16):803–808. doi: 10.1056/NEJM197304192881601. [DOI] [PubMed] [Google Scholar]
  6. Müller-Eberhard H. J., Götze O. C3 proactivator convertase and its mode of action. J Exp Med. 1972 Apr 1;135(4):1003–1008. doi: 10.1084/jem.135.4.1003. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Nicol P. A., Lachmann P. J. The alternate pathway of complement activation. The role of C3 and its inactivator (KAF). Immunology. 1973 Feb;24(2):259–275. [PMC free article] [PubMed] [Google Scholar]
  8. PILLEMER L., BLUM L., LEPOW I. H., ROSS O. A., TODD E. W., WARDLAW A. C. The properdin system and immunity. I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena. Science. 1954 Aug 20;120(3112):279–285. doi: 10.1126/science.120.3112.279. [DOI] [PubMed] [Google Scholar]
  9. Robinson M. G., Watson R. J. Pneumococcal meningitis in sickle-cell anemia. N Engl J Med. 1966 May 5;274(18):1006–1008. doi: 10.1056/NEJM196605052741806. [DOI] [PubMed] [Google Scholar]
  10. Soter N. A., Austen K. F., Gigli I. The complement system in necrotizing angiitis of the skin. Analysis of complement component activities in serum of patients with concomitant collagen-vascular diseases. J Invest Dermatol. 1974 Aug;63(2):219–226. doi: 10.1111/1523-1747.ep12679439. [DOI] [PubMed] [Google Scholar]
  11. Treatment and prevention of sickle-cell crisis. Lancet. 1971 Dec 4;2(7736):1255–1256. [PubMed] [Google Scholar]

Articles from British Medical Journal are provided here courtesy of BMJ Publishing Group

RESOURCES