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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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