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.