Intracorpuscular Red Cell Defect Masquerading as Valve Induced Hemolysis

Abstract

A case is described wherein hemolytic anemia, hemoglobinemia, and fragmented RBCs coexist with aortic valvular stenosis and insufficiency. The data obtained from “cross RBC survival” procedures indicated that hemolysis resulted primarily from an unidentified intracorpuscular defect despite several clinical and laboratory findings which suggested a cardiogenic mechanism as the etiology. This experience demonstrates the diagnostic usefulness of “cross RBC survival” procedures in cases where valvular disease, fragmented RBCs and intravascular hemolysis coexist, as well as the potential hazard of diagnosing valve-induced hemolysis by exclusion.