title: Comparison study of the eosin-5'-maleimide binding test, flow cytometric osmotic fragility test, and cryohemolysis test in the diagnosis of hereditary spherocytosis.
abstract: OBJECTIVE
  Current guidelines recommend the eosin-5'-maleimide (EMA) binding test and cryohemolysis test for screening for hereditary spherocytosis (HS), and the flow cytometric osmotic fragility (FC OF) test was recently developed to replace the classic OF test. We evaluatedthe performance of the EMA binding test, FC OF test, cryohemolysis test, and the hemoglobin (Hb)/mean corpuscular hemoglobin concentration (MCHC) ratio in the diagnosis of HS and assessed whether these tests reflect the clinical severity of HS.
  
  
  METHODS
  A total of 153 patients with anemia (33 with HS, 40 with autoimmune hemolytic anemia, 40 with anemia of chronic disease, and 40 with iron deficiency anemia [IDA]) and 140 healthy controls were enrolled, and the performance of the three tests was evaluated.
  
  
  RESULTS
  Both the EMA binding test (area under the curve [AUC], 0.996) and the FC OF test (AUC, 0.992) performed satisfactorily, but the cryohemolysis test (AUC, 0.723) performed significantly worse because of false positivity in patients with IDA. The Hb/MCHC ratio (P < .001) was able to reflect the clinical severity of HS.
  
  
  CONCLUSIONS
  Our results demonstrate that both the EMA binding and FC OF tests are useful as screening tests for the diagnosis of HS, but the cryohemolysis test has limited use due to its false positivity in IDA, with the Hb/MCHC ratio the most useful parameter for assessing the clinical severity of HS.
authors.affiliation: From the Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Sang Hyuk
authors.initials: SH
authors.lastname: Park
authors.affiliation: From the Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea; cjpark@amc.seoul.kr.
authors.forename: Chan-Jeoung
authors.initials: CJ
authors.lastname: Park
authors.affiliation: From the Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Bo-Ra
authors.initials: BR
authors.lastname: Lee
authors.affiliation: From the Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Young-Uk
authors.initials: YU
authors.lastname: Cho
authors.affiliation: From the Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Seongsoo
authors.initials: S
authors.lastname: Jang
authors.affiliation: Asan Institute for Life Science, Asan Medical Center, Seoul, Korea;
authors.forename: Nayoung
authors.initials: N
authors.lastname: Kim
authors.affiliation: Department of Pediatrics, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Kyung-Nam
authors.initials: KN
authors.lastname: Koh
authors.affiliation: Department of Pediatrics, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Ho-Joon
authors.initials: HJ
authors.lastname: Im
authors.affiliation: Department of Pediatrics, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea;
authors.forename: Jong-Jin
authors.initials: JJ
authors.lastname: Seo
authors.affiliation: Department of Pediatrics, Gyeongsang National University, School of Medicine, Gyeongsang Institute of Health Science, Jinju, Korea;
authors.forename: Eun Sil
authors.initials: ES
authors.lastname: Park
authors.affiliation: Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea; and.
authors.forename: Ji Won
authors.initials: JW
authors.lastname: Lee
authors.affiliation: Department of Pediatrics, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Keon Hee
authors.initials: KH
authors.lastname: Yoo
authors.affiliation: Department of Pediatrics, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Hye Lim
authors.initials: HL
authors.lastname: Jung
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D019411:Clinical Laboratory Techniques / Q000379:methods*; D003937:Diagnosis, Differential; D004801:Eosine Yellowish-(YS) / Q000031:analogs & derivatives* / Q000378:metabolism; D005189:False Positive Reactions; D005260:Female; D005434:Flow Cytometry; D006403:Hematologic Tests; D006461:Hemolysis; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009996:Osmotic Fragility; D012016:Reference Values; D012680:Sensitivity and Specificity; D013103:Spherocytosis, Hereditary / Q000175:diagnosis* / Q000378:metabolism; D055815:Young Adult
chemical_list: C033531:eosin maleimide; D004801:Eosine Yellowish-(YS)
keywords: Cryohemolysis test; EMA binding test; Flow cytometric osmotic fragility test; Hb/MCHC; Hereditary spherocytosis