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Coagulation Related Deficiencies of Early Components of C (C1, C4, C2) in Human Serum

Southern California Permanente Medical Group, Los Angeles, Calif.

Abstract

During the past 2 years we have studied the serum of a 48-year-old asymptomatic barber (R. G.) who has 0 CH₅₀ hemolytic C activity. Hemolytic assays of the individual serum components C1–C9 reveal 1 to 5% C1, C4, and C2 and normal activity of C3, C5, C6, C7, C8, and C9. C1 esterase inhibitor titers are only partially reduced and C3 PA protein is normal. Qualitative immunoelectrophoresis analysis of serum C1q, and Ouchterlony determinations of C1r and C1s appear normal as well. When R. G. plasma, with 10 units/ml heparin, EDTA, or citrate as anticoagulants, is used C activity is spared. CH₅₀ hemolytic activity is 60% of normal and C4, C2 are only partially reduced. C1 hemolytic activity in plasma is <1%. Since plasma rendered a protective effect on C4, C2, we wondered whether an abnormality in the coagulation sequence could account for this observation.