J Korean Assoc Pediatr Surg.  2017 Jun;23(1):1-4. 10.13029/jkaps.2017.23.1.1.

A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia

• Han WH
• Oh C
• Han JW
• Youn JK
• Kim HY
• Jung SE

• KMID: 2384422
• DOI: http://doi.org/10.13029/jkaps.2017.23.1.1

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.

Keyword

Extramedullary hematopoiesis; Congenital dyserythropoietic anemia; Anemia

MeSH Terms

Anemia
Anemia, Dyserythropoietic, Congenital*
Blood Transfusion
Erythroblasts
Hematologic Diseases
Hematopoiesis
Hematopoiesis, Extramedullary*
Humans
Parturition
Rare Diseases
Young Adult

Figure

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  Fig. 1 Abdominal MRI shows two homogeneous enhancing masses in both common iliac area (arrows). (A) Right side. (B) Left side.

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  Fig. 2 Technetium-99m RBC scintigraphy shows uptake in both common iliac area masses. (A) Right side. (B) Left side.

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  Fig. 3 The photomicrograph shows many polymorphous myeloid cells (H&E, ×400).

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