The Ewha Medical Journal
Ewha Womans University School of Medicine
Case Report

A Case of di Guglielmo Syndrome

So Kyung Park, Young Chin Chung, Chul Kyu Kim, Byoung Soo Kim
Corresponding author: Byoung Soo Kim. Dept. of Pediatrics, College of Medicine, Yonsei University, Korea.

Copyright ⓒ 1979. Ewha Womans University School of Medicine. 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.

Published Online: Jul 24, 2015

Abstract

A case of di Guglielmo syndrome in 9 year old boy was presented. This syndrome is characterized by excessive erythroid precursors with abnormal morphology both in the bone marrow and blood, and rare in childhood. He visited with the chief complaints of general weakness, weight loss, epistaxis and abdominal distension. Physical examination showed pallor, grade I-II/VI systolic murmur on apical area and marked abdominal distension with hepatosplenomegaly. we confirmed the patient by the peripheral smear and bone marrow aspiration.