Ringed Sideroblasts and Intramitochondrial Iron in Cases of Mechanical Hemolytic Anemia

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Abstract
Two patients who developed typical findings of mechanical hemolytic anemia following insertion of a Starr-Edwards aortic valve prosthesis are described. Partial correction of a diastolic leak around the valve significantly improved the anemia in 1 patient. A severe anemia dominated the clinical picture in the other patient as the prosthetic valve had become detached about most of its circumference. The cause of mechanical hemolytic anemia in patients with cardiovascular prosthetic material is excessive turbulence of blood probably directed against rigid prosthetic material and characteristically associated with a hemo-dynamic defect. A Prussian-blue stain of aspirated bone marrow revealed abnormal sideroblasts in both patients and numerous ringed forms in one case. Electron microscopy revealed a striking accumulation of intramitochondrial Fe in erythroid precursors of both patients. Both patients were hematologically normal before insertion of the prosthesis. There was no hematologic abnormality subsequently except mechanical hemolysis. Ringed sideroblasts and intramitochondrial accumulations of Fe are probably non-specific findings not necessarily reflecting a metabolic abnormality at the level of the red cell.