J Korean Med Sci.  2020 Aug;35(33):e279. 10.3346/jkms.2020.35.e279.

Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study

Affiliations
  • 1Department of Pediatrics, Keimyung University School of Medicine, Keimyung University Dongsan Medical Center, Daegu, Korea
  • 2Deparment of Pediatrics, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 3Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Cancer Research Institute, Seoul National University Children's Hospital, Seoul, Korea
  • 4Department of Pediatrics, Daegu Fatima Hospital, Daegu, Korea
  • 5Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea
  • 6Department of Pediatrics, Pusan National University School of Medicine, Pusan National University Children's Hospital, Yangsan, Korea
  • 7Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Gwangju, Korea
  • 8Department of Pediatrics, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
  • 9Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 10Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea
  • 11Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
  • 12Department of Pediatrics, School of Medicine, Kyungpook National University, Kyungpook National University Hospital, Daegu, Korea
  • 13Department of Pediatrics, Gyeongsang National University College of Medicine, Gyeongsang National University Hospital, Jinju, Korea
  • 14Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center Children's Hospital, Seoul, Korea
  • 15Department of Pediatrics, Dong-A University College of Medicine, Busan, Korea
  • 16Department of Pediatrics, Inha University Hospital, Incheon, Korea
  • 17Department of Pediatrics, Chosun University Hospital, Gwangju, Korea
  • 18Department of Pediatrics, Ewha Womans University College of Medicine, Ewha Womans University Seoul Hospital, Seoul, Korea
  • 19Department of Pediatrics, Center for Pediatric Cancer, National Cancer Center, Goyang, Korea
  • 20Department of Pediatrics, Jeju National University Hospital, Jeju National University School of Medicine, Jeju, Korea
  • 21Department of Pediatrics, Inje University College of Medicine, Busan Paik Hospital, Busan, Korea
  • 22Department of Pediatrics, Cha Bundang Medical Center, Cha University, Seongnam, Korea
  • 23Department of Pediatrics, University of Ulsan College of Medicine, Ulsan University Hospital, Ulsan, Korea
  • 24Department of Pediatrics, Hanyang University Seoul Hospital, Seoul, Korea
  • 25Department of Pediatrics, Korea University Ansan Hospital, Korea University College of Medicine, Ansan, Korea
  • 26Department of Pediatrics, Daegu Catholic University School of Medicine, Daegu, Korea
  • 27Department of Pediatrics, Kosin University College of Medicine, Kosin University Gospel Hospital, Busan, Korea

Abstract

Background
Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA.
Methods
We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey.
Results
A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia.
Conclusion
In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

Keyword

Congenital Hemolytic Anemia; Hereditary Spherocytosis; Hemoglobinopathies; Thalassemia; Glucose-6-phosphate Dehydrogenase Deficiency; Pyruvate Kinase
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