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KMID : 0366219840190020285
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Korean Journal of Hematology
1984 Volume.19 No. 2 p.285 ~ p.291
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A Case of Sideroblastic Anemia
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장진호/Jin Ho Jang
강만춘/손창학/박숙자/Man Choon Kang/Chang Hak Sohn/Sook Ja Park
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Abstract
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A case of Hereditary sideroblastic anemia is presented and the literatures of the case
are briefly reviewed. This 17 year-old girl was admitted to the department of IM of the
PMC on Apr, 22nd, 1984., because of sustained generalized weakness and exertional
dyspnea since childhood. But there was not profound distress in her life. She did not
have any bleeding tendency or purpuric lesion of the skin.
On the physical exammination general appearance was chronically ill and lethargic.
Face was pale, conjunctiva was severely anemic and sclera was not icteric. In the
cardiac ausculation Grade Ⅱ/Ⅵ functional murmur was noted in the Lt. 2nd ICS.
Abdomen was soft and flat, liver and spleen were not palpated. Stool was yellowish.
The peripheral blood smears showed severe anemia and typical dimorphism-
hypochromic microcytic and hyperchromic macrocytic-and poikilocytosis. And a few
nucleated red blood cells are found in the peripheral blood. Platelets and leukocytes were
relatively normal in number and shape. The bone marrow smears showed 90%
cellularity with marked erythroid hyperplasia consisting of mostly late stage in
development. M-E ratio was reversed by 1 : 2.6. Iron stain showed many ringed
sideroblasts, 41% of normoblasts, and increased amounts of hemosiderin particles within
histiocytes. PAS stains are positive to reaction in many normoblasts.
After a high dosage of pyridoxine administration, anemia was not corrected. So we
report this rare case of hereditary sideroblastic anemia.
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