Refractory Sideroblastic Anemia

Abstract

Ten adults with "refractory" anemia and hyperplastic marrow were studied over a period of four years. Disturbance in iron metabolism at a cellular level was manifested by the finding of hemoglobin-poor nucleated red cells in the marrow in association with large amounts of coarse siderotic granules in varying degrees in all cases. The anemia was primarily due to "ineffective erythropoiesis". Despite the fact that four patients died without evidence of leukemia transformation, the common finding of disturbed iron metabolism as well as cytoplasmic PAS-positive material, emphasized their similarity to chronic Di Guglielmo''s Syndrome in which leukemia often occurs. In those cases in which leukemia either developed or appeared imminent, a history of exposure to toxic substances accompanied by definite qualitative changes in myeloid elements suggests their prognostic significance in other cases of refractory anemia with hyperplastic marrow.