Idiopathic Dyserythropoietic Jaundice

Abstract

Abstract 1. The term "dyserythropoietic jaundice" is used to designate a peculiar abnormality characterized by marked overproduction of bilirubin and associated with unconjugated hyperbilirubinemia and great excesses of fecal urobilinogen. Labeling with N15 glycine revealed that these excesses were mainly unrelated to destruction of mature circulating red cells, which had only a slightly shortened life span. 2. The hyperplastic, normoblastic bone marrow exhibited considerable phagocytosis of red cells and normoblasts and marked hemosiderosis with many iron-laden phagocytes. Siderocytes were relatively rare in the peripheral blood, which also exhibited consistent reticulocytosis, 3-10 per cent, but normal erythrocyte porphyrin concentrations. There was increased plasma iron turnover but decreased appearance of Fe59 in the circulating red cells. 3. The possibility is considered that there is a basic abnormality in hemoglobin synthesis in the normoblasts, with excessive production of hemoglobin or heme which is then converted to bilirubin either within the normoblast or after excretion into the blood. The observed phagocytosis of normoblasts and erythrocytes may be a secondary rather than basic disturbance of the disease. 4. This case appears to represent a unique form of chronic jaundice due to a remarkable overproduction of bilirubin without evidence of hemolysis in the ordinary sense. The extent to which this is related to intramedullary destruction of young red cells or to a basic abnormality in hemoglobin synthesis in the normoblasts, as above, cannot be determined. It is considered less likely that the overproduction is due to a true shunt—that is, bilirubin formation from porphyrin precursors. The marked dyserythropoiesis suggests that the overproduction of bilirubin was medullary rather than hepatic, although the latter is not excluded.