A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia.
10.13029/jkaps.2017.23.1.1
- Author:
Won Ho HAN
1
;
Chaeyoun OH
;
Ji Won HAN
;
Joong Kee YOUN
;
Hyun Young KIM
;
Sung Eun JUNG
Author Information
1. Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea. chaeyoun.o@gmail.com
- Publication Type:Case Report
- Keywords:
Extramedullary hematopoiesis;
Congenital dyserythropoietic anemia;
Anemia
- MeSH:
Anemia;
Anemia, Dyserythropoietic, Congenital*;
Blood Transfusion;
Erythroblasts;
Hematologic Diseases;
Hematopoiesis;
Hematopoiesis, Extramedullary*;
Humans;
Parturition;
Rare Diseases;
Young Adult
- From:Journal of the Korean Association of Pediatric Surgeons
2017;23(1):1-4
- CountryRepublic of Korea
- Language:Korean
-
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.
