Erythremia with special reference to sideroblastic anemia

An autopsy case of erythremia with sideroblastic tumor cell proliferation is described. A 60-year-old man was admitted to the hospital due to general fatigue and anorexia. Bone marrow aspiration revealed abnormalities in erythropoiesis (megaloblasts, 4%; sideroblasts, 84%; ring-formed, 39%, and PAS-positive, 5%). Therapy was directed to pulmonary tuberculosis. Anemia was not improved despite repeated whole blood and platelet transfusions. Serum iron and percentage saturation of the total iron-binding capacity rose during the course. Administration of vitamin B12, B6 or folic acid was inefffective. INAH was replaced by its derivative, IHMS, during the course, but the population of sideroblasts especially of ring-sideroblasts was invariably large (78%-100% and 39%-65% for total sideroblasts and ring-sideroblasts, respectively). He died with increasing abdominal pain and jaundice after three months' hospitalization. Main autopsy findings were: diffuse proliferation of atypical erythroblasts in the bone marrow, systemic lymph nodes, liver, spleen and kidneys. Most of the cells positively stained with iron. Tuberculosis of lungs with cavity formation. Discussion is focussed on the relationship between erythremia and sideroblastic anemia.