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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. 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. 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. 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. © 2020 The Korean Academy of Medical Sciences.


Ye Jee Shim, Hye Lim Jung, Hee Young Shin, Hyoung Jin Kang, Jung Yoon Choi, Jeong Ok Hah, Jae Min Lee, Young Tak Lim, Eu Jeen Yang, Hee Jo Baek, Hyoung Soo Choi, Keon Hee Yoo, Jun Eun Park, Seongkoo Kim, Ji Yoon Kim, Eun Sil Park, Ho Joon Im, Hee Won Chueh, Soon Ki Kim, Jae Hee Lee, Eun Sun Yoo, Hyeon Jin Park, Jun Ah Lee, Meerim Park, Hyun Sik Kang, Ji Kyoung Park, Na Hee Lee, Sang Kyu Park, Young Ho Lee, Seong Wook Lee, Eun Jin Choi, Seom Gim Kong, Red Blood Cell Disorder Working Party of the Korean Society of Hematology. Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997-2016: a Nationwide Retrospective Cohort Study. Journal of Korean medical science. 2020 Aug 24;35(33):e279

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PMID: 32830468

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