Intracorpuscular red cell defect masquerading as valve induced hemolysis.

he occasional occurrence of chronic intravascu lar hemolysis following cardiac prosthetic sur gery is well documented.1 In this syndrome the pathophysiologic sequence of events has been postulated to occur as follows: turbulence from val vular deformity fragments erythrocytes and induces intravascular hemolysis. If severe, the resulting anemia increases both cardiac output and turbu lence. Long term sequelae, besides anemia, are renal hemosiderosis and iron deficiency through unrelenting urinary iron 1055.2 Recently, attention has been focused on an ap parently identical syndrome occurring in nonsur gical patients with valvular heart disease.8 A patient with aortic valvular disease, fragmented erythrocytes, and intravascular hemolysis has been studied. Data indicated an intracorpuscular defect of unknown identity rather than the valvular lesion as the primary etiologic mechanism. This experi ence is reported to emphasize that a commonly em ployed approach to differentiating between these mechanisms could eventuate in an unwarranted thoracotomy. Routine hematologic studies were performed using stand ard methods. The following studies were performed accord ing to published methods: incubation hemolysis, RBC mechanical fragility, RBC life span, glutathione reductase, pyruvate kinase, RBC osmotic fragility, cold agglutination, and Donath-Lanclsteiner cold hemolysis, acid hemolysis, fe tal hemoglobin, RBC sequestration, â€oe¿¿ sugar water†• test, â€oe¿¿ Heinz body†• prep, glucose 8 phosphate dehydrogenase (G-6-PD) activity, and serum vitamin B12. Red cell fragments (schistocytes) were examined on stained smears and enumerated according to the criteria of Tuffy et al.4 Hemosiderin in body tissues and fluids was demon strated by prussian blue staining. The following studies were performed according to pub lished methods: serum complement, haptoglobin, serum iron, iron binding capacity, fecal urobilinogen, and porphobilin ogen. Harlico kit No. 64208 was used in testing for urinary heavy metals. Specimens were obtained twice daily: at dawn prior to patient's arising and at 1 PM after ambulation, stair climb ing, and dining. Following atraumatic venipuncture, blood was drawn into a plastic syringe through a No. 19 needle, was placed in a siliconized tube containing a drop of hepa tin, and plasma was separated immediately by centrifuga