Scleroderma and Microangiopathic Hemolytic Anemia

Microangiopathic hemolytic anemia was found in 7 of 20 patients with systemic scleroderma, an indication that this complication is not uncommon. Since only four had severe hypertension, pressures in the "malignant " range seem not to be necessary for the development of the anemia. All seven patients had renal arterial fibrin deposits, which supports the concept that erythrocyte fragmentation results from contact with intravascular fibrin. MICROANGIOPATHIC HEMOLYTIC ANEMIA is charac-terized by the presence of fragmented and deformed erythrocytes in the peripheral blood; usually, a nega-tive Coombs ' reaction; and, often, thrombocyto-penia. Fibrin deposition is found in the vessels of the kidney and elsewhere. It is thought that the changes in the erythrocytes result from damage in-flicted on them by contact with intravascular deposits of fibrin (1). This anemia has been described in association with a variety of diseases: malignant hypertension, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, renal allograft rejection, and pre-eclampsia (1, 2). It has been suggested that the formation of fibrin deposits on damaged endothelium may be an important cause of the arterial lesions in these diseases (3). Because of the great similarity of the vascular changes in scleroderma to those seen in the above conditions, especially in the kidney, it seems reasonable to expect to find renal arterial fibrinoid deposits and microangiopathic hemolyti