A 60-year-old man was referred to our institution for evaluation of anemia. He had not received medical examination or treatment for years and had no family history of hematological disorders. He used a moderate amount of alcohol daily (75–100 g ethanol per day) for more than a decade. Laboratory examination at the first visit showed white blood cells, 2,900/mm (71 % neutrophils, 20 % lymphocytes, 6 % monocytes, 3 % eosinophils, 1 % basophils); hemoglobin concentration, 7.6 g/dl; platelet count, 301,000/mm, MCV, 106 fl; MCH, 36.2 pg; MCHC, 34.2 %; reticulocyte count, 3.4 %; AST, 60 IU/l; ALT, 84 IU/l; LDH, 225 IU/l; direct bilirubin, 0.15 mg/dl; indirect bilirubin, 2.31 mg/dl; C-reactive protein 0.31 mg/dl; serum iron, 232 lg/dl; UIBC, 16 lg/dl; ferritin, 3,278 ng/ ml; and haptoglobin, 16 mg/dl. Serum concentrations of vitamins B6 and B12 and of folic acid were normal, and HBs antigen, HBs antibody, and HCV antibody were negative. Bone marrow (BM) examination revealed hypercellular marrow (245,000/mm) dominated by erythroblasts with megaloblastic changes. Myeloblasts included 1 % nucleated cells, and there was no apparent myeloid or megakaryocytic dysplasia. On iron staining, most erythroblasts were ringed sideroblasts (Fig. 1a). Chromosomal analysis found normal male karyotype. A tentative diagnosis of refractory anemia with ringed sideroblasts subtype of myelodysplastic syndrome (MDSRARS) was made. Metenolone acetate (10 mg/day) was initiated, but hemoglobin levels did not improve (Fig. 1b). The possibility of X-linked sideroblastic anemia (XLSA), a congenital disorder that is occasionally diagnosed later in life [1], was considered, and pyridoxal phosphate hydrate (90 mg/day) was administered orally, but was ineffective (Fig. 1b). In addition to very high serum ferritin (3.278 ng/ ml) and elevated serum transferrin saturation (93.5 %), mild hepatic dysfunction (AST 60 IU/l, ALT 84 IU/l) and mild hepatosplenomegaly with markedly high density of the liver on CT was observed at presentation (Fig. 1c). Thus, this patient was considered to have iron overload (IO), despite the fact that he had never been transfused, was negative for HCV infection, and had no signs of chronic inflammation. Neither glucose intolerance nor decreased cardiac function was observed. After 1 year from the first visit, despite significant reduction of alcohol intake, serum ferritin levels remained high, and the abnormalities in liver function remained unchanged (Fig. 1b). Considering the possibility that sustained IO in the liver may lead to hepatic cirrhosis and/or hepatocellular carcinoma [2], we initiated administration of an oral iron chelating agent (deferasirox 1,000 mg/day). Serum ferritin decreased steadily, falling to less than 500 ng/ ml in 19 months (Fig. 1b). AST and ALT also normalized, and the density of the liver on CT decreased from 100 to 70 HU. No hematological improvement was observed, either in the peripheral blood (PB) or in the BM. Thus, the clinical picture of this patient consisted of an unusual combination of sideroblastic anemia and clinically H. Ohashi H. Nagai Division of Hematology, National Hospital Organization Nagoya Medical Center, Nagoya, Japan