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KMID : 0366219760110010039
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Korean Journal of Hematology
1976 Volume.11 No. 1 p.39 ~ p.45
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A Case Report of Congenital Pure Red Cell Anemia
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김광평/Gwang Pyung Kim
이경수/차상덕/이두봉/김선무/이종무/Kyong Su Lee/Sang Duk Cha/Du Bong Lee/Sun Moo Kim/Chong Moo Lee
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Abstract
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Congenital pure red cell anemia which occurs primarily in young infants is a rare
disease entity and characterized by a chronic progressive anemia confined to a failure of
erythropoiesis without an equivalent depression of the white blood cells or platelets.
This paper presented a case of congenital pure red cell anemia in a 8 months old
male who had become gradually pale since the birth.
Four transfusions were required until 8 months of age when he was hospitalized
because of severe pallor, intractable diarrhea, irritability and respiratory distress.
Physical examination revealed a markedly pale infant with wide-set eyes and
brachycephaly. The liver was moderately enlarged and a heart murmur was presented.
On laboratory findings, the hemoglobin, hematocrit and reticulocyte count were
extremely low, but normal white blood cell and platelet counts. The peripheral smear
showed a characteristic normochromic, normocytic anemia.
In the bone marrow smear the red cell precursors were decreased markedly, resulting
in myeloid-erythroid ratios of 100:1. The cells of both myeloid and erythroid series were
normal in structure.
He was treated with transfusions and corticosteroid until discharge on the 22nd
hospital day. There were some clinical improvement and increasing tendency of
reticulocyte counts.
The authors reviewed the referential literatures briefly.
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