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KMID : 0605920170230010001
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Journal of Korean Association of Pediatric Surgeons
2017 Volume.23 No. 1 p.1 ~ p.4
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A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia
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Han Won-Ho
Oh Chae-Youn
Han ji-Won
Youn Joong-Kee
Kim Hyun-Young
Jung Sung-Eun
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Abstract
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It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.
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KEYWORD
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Extramedullary hematopoiesis, Congenital dyserythropoietic anemia, Anemia
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