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KMID : 0366219740090010019
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Korean Journal of Hematology
1974 Volume.9 No. 1 p.19 ~ p.25
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Congenital Hypoplastic Anemia (Blackfan-Diamond Syndrome)
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박성원(朴聖媛)/Sung Won Park
신상만(申相滿)/신애라(申愛羅)/고광욱(高光昱)/홍창의(洪彰義)/Sang Man Shin/Aira Shin/Kwang Wook Ko/Chang Yee Heng
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Abstract
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Congenital hypoplastic anemia is a rare discase in young infants. More than 100 cases
have been reported since the disease was described over 35 years ago. It is considered
to be a primary disorder of the bone marrow, characterized by relative
depression of red blood cell production. The anemia is normochromic and normocytic
in type and is associated with a reticulocytopenia of less than 1 percent.
One case of congenital hypoplastic anemia was observed in a 13 months old male
patient.
No other congenital defect was observed. Good hematologic response was obtained by
corticosteroid therapy
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