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Han, Oh, Han, Youn, Kim, and Jung: A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia
Case Report

Journal of the Korean Association of Pediatric Surgeons 2017; 23(1): 1-4.

Published online: 26 June 2017

DOI: https://doi.org/10.13029/jkaps.2017.23.1.1

A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia

Won-Ho Han, Chaeyoun Oh, Ji-Won Han, Joong-Kee Youn, Hyun-Young Kim, Sung-Eun Jung

Department of Pediatric Surgery, Seoul National University Children's Hospital, Seoul, Korea.

Correspondence: Chaeyoun Oh, Department of Pediatric Surgery, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel: +82-2-2072-4252, Fax: +82-2-766-3975, chaeyoun.o@gmail.com

Received 21 July 2016       Revised 4 November 2016       Accepted 27 December 2016

Copyright © 2016 by the Korean Association of Pediatric Surgeons

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) 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

Figures and Tables

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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Notes

CONFLICTS OF INTEREST No potential conflict of interest relevant to this article was reported.

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