Deficiency of factor B of the complement system in sickle cell anaemia.
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.
D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000276:immunology*; D001424:Bacterial Infections / Q000150:complications; D003176:Complement C3 / Q000172:deficiency*; D003165:Complement System Proteins / Q000172:deficiency*; D004792:Enzyme Precursors / Q000378:metabolism*; D005260:Female; D005916:Globulins / Q000378:metabolism*; D006023:Glycoproteins / Q000172:deficiency*; D006801:Humans; D008297:Male; D010587:Phagocytosis
D003176:Complement C3; D004792:Enzyme Precursors; D005916:Globulins; D006023:Glycoproteins; D003165:Complement System Proteins