Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006

Cho, Hee Soon; Hah, Jeong Ok; Kang, Im Ju; Kang, Hae-Lim; Kwak, Jae Yong; Koo, Hong Hoe; Kook, Hoon; Kim, Byoung Kook; Kim, Soon Ki; Kim, Seung Taik; Kim, Ji Yoon; Kim, Chul Soo; Ghim, Thad T.; Kim, Heung Sik; Park, Sang Gyu; Park, Seon Yang; Park, Jun Eun; Bang, Soo Mee; Seo, Jong Jin; Suh, Chang In; Sohn, Sang Kyun; Shin, Ho Jin; Shin, Hee Young; Ahn, Hyo Sup; Oh, Dong Young; Yoo, Eun Sun; Lyu, Chuhl Joo; Yoon, Sung Soo; Lee, Kun Soo; Lee, Kwang Chul; Lee, Kee Hyun; Lee, Soon Yong; Lee, Young Ho; Lee, Jung Ae; Lee, Jong Seok; Lim, Young Tak; Lim, Jae Young; Im, Ho Joon; Jeong, Dae Chul; Chong, So Young; Chung, Joo Seop; Jung, Hye Lim; Cho, Goon Jae; Jo, Deog Yeon; Jin, Jong Youl; Choi, Eun Jin; Hyun, Myung Soo; Hwang, Pyoung Han

doi:10.5045/kjh.2007.42.3.197

Cited by 17 publications

(24 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The frequency of these symptoms was similar to that reported in the 1997-2006 survey [4]. The symptoms and signs of patients with hemoglobinopathies were asymptomatic, anemia, and jaundice.…”

Section: Resultssupporting

confidence: 86%

“…A standard diagnostic approach for the accurate diagnosis of HHA is warranted since 2 nationwide epidemiologic and clinical studies in Korea, conducted from 1981 to 1990 [3] and from 1997 to 2006 [4], respectively, showed an increase in the prevalence of hemoglobinopathies and RBC enzymopathies. To address this, a working party for HHA was established by the Korean Hematology Association.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 2013

Self Cite

View full text Add to dashboard Cite

BackgroundThe 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.MethodsInformation 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.ResultsIn 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.ConclusionThe 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.

show abstract

Section: Resultssupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

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

et al. 2013

Self Cite

View full text Add to dashboard Cite

show abstract

“…In a review of Korean literature on HHA, we found 15 case reports on HE, including our case [1, 16]. Red-cell-membrane defects were the most common (88.6%) in HHA.…”

mentioning

confidence: 96%

“…In Korea, HE is the cause of 1.4% (6/431) cases of hereditary hemolytic anemia (HHA) [1]. HE causes mechanical weakness or fragility of the erythrocytic membrane skeleton because of defects in spectrin or protein 4.1.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Spectrin Tunis (Sp alpha^I/78) in a Korean Family with Hereditary Elliptocytosis

et al. 2013

View full text Add to dashboard Cite

Flow cytometric detection of erythrocyte osmotic fragility

Won

Suh

2008

Cytometry Part B Clinical

View full text Add to dashboard Cite

Background: Hereditary spherocytosis (HS) is the most common cause of inherited chronic hemolysis.The various tests developed for HS screening have many shortcomings. The purpose of this study was to develop a new, simple, reliable test using flow cytometry.Methods: In this new test, deionized water, a hemolysis-inducing agent, is spiked to a red cell suspension during acquisition, and the count of red cells is measured sequentially in real-time using flow cytometry. In this study, the optimal parameters for this test were established, and the discriminatory power of the adopted protocol was verified among several groups with various degrees of osmotic fragility (OF).Results: The established protocol successfully discriminated between blood analyzed immediately and blood stored for 24 h (P < 0.00001). With healthy individuals as a reference, the OF measured by this protocol was significantly decreased in patients with iron deficiency anemia (P = 0.030) and was significantly increased in HS patients (P = 1.1 × 10 29 ). This new test found all 11 HS patients to have increased OF.Conclusions: This new test was verified to be simple, quantitative, objective, and cost-effective for the measurement of OF. We suggest this test as another effective approach for HS screening, although its assay performance will require further verification through more clinical trials. q 2008 Clinical Cytometry

show abstract

Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006

Cited by 17 publications

References 6 publications

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

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

Spectrin Tunis (Sp alpha^I/78) in a Korean Family with Hereditary Elliptocytosis

Flow cytometric detection of erythrocyte osmotic fragility

Contact Info

Product

Resources

About

Hereditary Hemolytic Anemia in Korea: a Retrospective Study from 1997 to 2006

Cited by 17 publications

References 6 publications

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

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

Spectrin Tunis (Sp alphaI/78) in a Korean Family with Hereditary Elliptocytosis

Flow cytometric detection of erythrocyte osmotic fragility

Contact Info

Product

Resources

About

Spectrin Tunis (Sp alpha^I/78) in a Korean Family with Hereditary Elliptocytosis