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KMID : 0366219670020010045
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Korean Journal of Hematology
1967 Volume.2 No. 1 p.45 ~ p.50
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Case Report of Di Guglielmo Syndrome (Chronic Erythremic Myelosis)
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송희승(宋熙昇)/Hee Sung Song
이범홍(李凡弘)/임도수(林道洙)/정경태(鄭敬泰)/김관엽(金官曄)/김상인(金相仁)/지제근(池堤根)/Bum Hong Lee/Do Soo Lim/Kyung Tae Jung/Kwan Youp Kim/Sang In Kim/Jae Keun Chi
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Abstract
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Clinical and hematological studies on a case of Di Guglielmo syndrome (Erythremic
myelosis) is presented.437 year old Korean male was admitted to the Seoul National
University Hospital with the complaints of general weakness, vertigo, headache,
intermittent melena, epistaxis and gingival bleeding. Physical examinations on admission
reveals pallor, hepatosplenomegaly and percussion pain on the sternal area. Peripheral
blood examinations show many polychromatophilic and orthochromatophilic normoblasts,
abnormal form of normoblasts, marked anisocytosis and poikilocytosis with
polychromasia and several immature gramlocytes. Bone marrow aspiration reveals
erythroid hyperplasia (L,/E ratio 1 : 4) with many dysplastic and/or anaplastic
normoblasts. The erythropoietic indices calculated from the plasma iron turnover and
L/E ratio of the bone marrow were higher than the erythropoietic indices calculated
from the iron utilization and the life span of the red cells, thus indicating "ineffective"
erythropoiesis of the marrow. We ruled out pernicious anemia by the administration of
Vitamin B12 for 5 weeks. The treatments were chiefly aimed to control
the anemia and bleeding tendency with fresh whole blood transfusion and prednisolone.
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KEYWORD
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