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KMID : 0384119940140020135
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Korean Journal of Clinical Pathology
1994 Volume.14 No. 2 p.135 ~ p.141
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A Case of Congenital Pure Red Cell Anemia
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Abstract
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Congential pure red cell aplasia or Diamond Blackfan anemia is agenerative anemia of childhood. This condition is relatively rare and begins in early in early in early infancy. The basic abnormality is characterised by a chronic progressive
anemia
confined to a failure of erythropoiesis without an equivalent depression of the white blood cells and platelets. Many theories have been advanced concerning the etiology of it, but it is not clear. Treatment consists of blood transfusion,
splenectomy,
corticosteroid, bone marrow transplantation and recombinant human interleukin-3, but long term prognosis remains uncertain.
Recently we have had an opportunity to observe an infant with Diamond-Blackfan syndrome. He was admitted to our hospital because of extremely pale and waxy white skin for 4 weeks. In the bone marrow, the red cell precursors were markedly
decreased
and
estimated M:E ratio was about 50:1. Hematologic improvements was followed by administration of prednisolone and M:E ratio was about 2.3:1 on the follow up bone marrow study done at 17 months later. So we report this case with brief review of
literature.
(Korean J Clin Pathol 1994 ; 14(2) : 135-41)
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