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A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia
선천성 적혈구 생성 이상 빈혈과 관련되어 발생한 골수 외 조혈 증례 보고
JKAPS 2017 Jun;23(1):1-4
Published online June 30, 2017
Copyright © 2017 Korean Association of Pediatric Surgeons.

Won-Ho Han, Chaeyoun Oh, Ji-Won Han, Joong-Kee Youn, Hyun-Young Kim, Sung-Eun Jung
한원호, 오채연, 한지원, 윤중기, 김현영, 정성은

Department of Pediatric Surgery, Seoul National University Children’s Hospital, Seoul, Korea
서울대학교 어린이병원 소아외과
Received July 21, 2016; Revised November 4, 2016; Accepted December 27, 2016.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
 Abstract
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.
Keywords : Extramedullary hematopoiesis, Congenital dyserythropoietic anemia, Anemia

 

June 2017, 23 (1)