Malignant Hypertension and Red Cell Fragmentation Syndrome

Abstract

A red-cell fragmentation syndrome was previously described in various disease states including malignant hypertension and also in association with defective intracardiac prosthetic devices. Mechanical disruption of the red cell by diseased endothelium or an intracardiac prosthesis has been proposed to account for the hematologic derangement. A fatal case of malignant hypertension, rapidly progressive renal failure, and a red-cell fragmentation syndrome is described in a 48 year old Negro woman. The patient had been in apparent good health until just prior to her hospital admission. Immediately following hospitalization she developed complete anuria and remained so until death. Throughout her course the peripheral blood film contained large numbers of fragmented cells (schistocytes), burr and helmet cells. Thrombocytopenia, severe anemia, and reticulocytosis were prominent features. Serial renal biopsies demonstrated progressive vascular lesions of malignant nephrosclerosis. However, the vascular lesions appeared insignificant when red-cell fragmentation and thrombocytopenia were severe. To explain the erythrocyte fragmentation on purely a mechanical basis therefore appears inadequate.