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Images in Clinical Practice

Indian Pediatrics 2001; 38: 926  

Sideroblastic Anemia


A 10-year-old boy presented with severe pallor and breathlessness. He had a past history of similar illness two years prior to the current episode when he had also required blood transfusion. After the first episode, he had to be transfused twice more. On examina-tion, he had severe pallor, no jaundice, no lymphadenopathy and no bleeds. He had hyperpigmentation of the skin (especially over the perioral area and also on the palms) and mild malar prominence. There was moderate hepatosplenomegaly. The child was in cardiac failure. Investigation revealed a Hb of 3 g/dl, reticulocyte count <1% and TLC 9000 (P64, L36). His peripheral smear showed microcytic hypochromic anemia. Bone marrow aspirate showed erythroid hyperplasia with ring sideroblasts on Prussian Blue staining (Fig. 1) Liver biopsy showed evidence of hemochromatosis. A diagnosis of sideroblastic anemia was made and he was treated with blood transfusions and started on high doses of Pyridoxine (300 mg/day). Unfortunately, he failed to show a significant reticulocyte response.

Fig.1. Bone marrow aspirate showing ring sideroblast (arrow). The nucleus is surrounded by iron containing granules stained with Prussian Blue.

Janaki Menon,
Lulu Mathews
,
Department of Pediatrics,
Medical College, Thrissur,
Kerala, India.

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