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Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology

Authors
 Eun Sil Park  ;  Hye Lim Jung  ;  Hee-Jin Kim  ;  Sung Sup Park  ;  Soon Hwan Bae  ;  Hee Young Shin  ;  Sang Hoon Song  ;  Kyung-Nam Koh  ;  Chuhl Joo Lyu  ;  Young Tak Lim  ;  Dong Kyun Han  ;  Jeong Ok Hah 
Citation
 BLOOD RESEARCH, Vol.48(3) : 211-216, 2013 
Journal Title
 BLOOD RESEARCH 
ISSN
 2287-979X 
Issue Date
2013
Keywords
Congenital hemolytic anemia ; Congenital nonspherocytic anemia ; Hereditary spherocytosis ; Thalassemia
Abstract
BACKGROUND: The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from a survey on the prevalence and characteristics of patients diagnosed with HHA in Korea from 2007 to 2011. METHODS: Information on patients diagnosed with HHA in Korea and their clinical and laboratory results were collected using a survey questionnaire. Globin gene and red blood cell (RBC) enzyme analyses were performed. In addition, we analyzed data collected by pediatricians. RESULTS: In total, 195 cases of HHA were identified. Etiologies identified for HHA were RBC membranopathies, hemoglobinopathies, and RBC enzymopathies, which accounted for 127 (64%), 39 (19.9%), and 26 (13.3%) cases, respectively. Of the 39 patients with hemoglobinopathies, 26 were confirmed by globin gene analysis, including 20 patients with β-thalassemia minor, 5 patients with α-thalassemia minor, and 1 patient with unstable hemoglobin disease. CONCLUSION: The number of patients diagnosed with hemoglobinopathies and RBC enzymopathies has increased considerably since the previous survey on HHA in Korea, dated from 1997 to 2006. This is likely the result of improved diagnostic techniques. Nevertheless, there is still a need for more sensitive diagnostic tests utilizing flow cytometry and for better standardization of test results to improve the accuracy of diagnosis of RBC membranopathies in Korea. Additionally, more accurate assays for the identification of RBC enzymopathies are warranted.
Files in This Item:
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DOI
10.5045/br.2013.48.3.211
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Lyu, Chuhl Joo(유철주) ORCID logo https://orcid.org/0000-0001-7124-7818
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/88779
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