FAMILIAL HYPOCHROMIC ANEMIA ASSOCIATED WITH POSTSPLENECTOMY ERYTHROCYTIC INCLUSION BODIES

Abstract

Abstract A case is presented of familial, hypochromic, microcytic anemia, associated with the appearance of siderocytes in the peripheral blood following splenectomy. The medical literature of the recent past focuses attention on the clinical recognition of inclusion bodies, but their origin and significance have not been completely clarified. Rundles and Falls4 were the first to demonstrate them in hereditary hypochromic microcytic anemia, and in addition they have been shown to appear in acquired hemolytic anemia,2, 3 Banti’s syndrome,1 lead poisoning,3, 15 and in hemochromatosis, bacterial toxemias, industrial solvent poisoning, sickle cell anemia and acholuric jaundice.15 It is probable that the anemia in the case under discussion may have been due to some defect in iron metabolism. Neither the subject nor his maternal half-brother were demonstrated to have any objective evidence of hemolysis, and neither revealed reticulocytosis, despite intensive iron and liver therapy, a point in favor of poor utilization of iron. Furthermore, marked hemosiderosis was an outstanding feature of both cases signifying that at least one form of storage iron was available but not utilized. The significance of inclusion bodies within erythrocytes is discussed and a classification of inclusion bodies is offered. A final statement regarding the nature of iron granules within red cells must await further research. When present within the erythrocytes of the peripheral blood or in the erythroid series of the bone marrow, they probably are the result of faulty iron metabolism either due to some inherent defect or secondary to the action of some noxious agent. Their prognostic significance is obscure, but this is probably related to the severity of the disease process of which they are an expression.