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Hereditary C5 Deficiency in Man

III. Studies of Hemostasis and Platelet Responses to Zymosan^1,2,

From the Department of Medicine, University of Rochester School of Medicine and Dentistry, and the Rochester General Hospital, Rochester, New York 14621

Abstract

Platelet-rich-plasma from two hemostatically normal individuals, genetically lacking the fifth component of complement (C5), failed to exhibit normal platelet aggregation, or serotonin release, in the presence of zymosan. This abnormality was found to reside in the C5D plasma rather than in the platelets as demonstrated by the inability of the deficient plasma to activate zymosan for the aggregation of washed normal platelets. The defect could be corrected by the addition of normal plasma, normal serum, or highly purified human C5.

A plasma abnormality similar to that found in the C5D individuals was also noted in plasmas deficient in C3, C6, and C7; whereas C8 plasma D behaved normally. These data suggest that this platelet reaction requires late acting C components, perhaps as the
complex, bound to the zymosan particles.

Footnotes

¹ This work was supported, in part, by United States Public Health Service Research Grants AM-09810 and AI-12568 and United States Public Health Service Training Grant AM-05133.

² Presented at the Sixth International Complement Workshop, Sarasota, Florida, November 23 to 25, 1975.

³ Address reprint requests to: Robert T. Breckenridge, M. D., Rochester General Hospital, 1425 Portland Avenue, Rochester, New York.