Abstract
Summary
The in vitro acid hemolysis of red cells from patients with paroxysmal nocturnal hemoglobinuria (PNHE) is intensified by reducing the ionic strength to 0.042. The ability of PNHE to form an intermediate complex by direct reaction with isolated C′3a (PNHEC'3a) is not affected by decreasing the ionic strength; the hemolysis of PNHEC'3a is inhibited by reduction in ionic strength. The effects of low ionic strength and first complement component (C'1) esterase or a C'1 activator upon the enhancement of PNHE lysis are not additive, which suggests that these factors share a common mechanism. The effects of ionic strength on PNHE hemolysis are similar to those observed in other complement dependent hemolytic systems.
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