Circulating immune complexes and complement levels in hemophilic children.
Immune complexes and complement levels were assayed in sera from a group of 69 hemophilic children. Using the Raji cell radioimmune assay and the C1q binding assay, abnormally high levels of circulating immune complexes were rarely found in the group of hemophiliacs which did not differ statistically from the control population. These results do not exclude the presence of low and transient levels of immune complexes in the circulation, but indicate that hemophiliacs are not exposed to increased immune complex loads similar to those found in immune complex diseases. By contrast frequent abnormalities of the complement system were found. Complement levels were elevated in a large percentage of patients, reaching statistical significance for C3, C4, C5, Factor B and Properdin. Levels of the C3 breakdown product C3d were significantly raised suggesting intravascular complement activation. The significance of these abnormalities is discussed in relation to perfusion of Factor VIII preparations.
D000293:Adolescent; D000328:Adult; D000936:Antigen-Antibody Complex / Q000032:analysis*; D002648:Child; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003182:Complement C5 / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis*; D006467:Hemophilia A / Q000276:immunology*; D002836:Hemophilia B / Q000276:immunology*; D006801:Humans
D000936:Antigen-Antibody Complex; D003176:Complement C3; D003181:Complement C4; D003182:Complement C5; D003165:Complement System Proteins; D011415:Complement Factor B