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We examined in a large cohort of homozygous Hb E if the level of Hb A<jats:sub>2<\/jats:sub> is helpful.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 592 subjects with homozygous Hb E were recruited from ongoing thalassemia screening program. Additionally, five couples at risk of having fetuses with Hb Bart's hydrops fetalis who were homozygous Hb E were also investigated. Hb analysis was performed using capillary electrophoresis system. Globin genotypes were defined by <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> analysis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Subjects were classified into four groups including pure homozygous Hb E (n=532), homozygous Hb E\/\u03b1<jats:sup>0<\/jats:sup>\u2010thalassemia (n=48), Hb Constant Spring <jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content> Bart's disease (n=8), and Hb <jats:styled-content style=\"fixed-case\">EE<\/jats:styled-content> Bart's disease (n=4). The levels of Hb A<jats:sub>2<\/jats:sub> were found, respectively, to be 4.97\u00b10.69, 6.64\u00b11.02, 4.86\u00b10.87, and 7.60\u00b11.04%. Among five couples at risk, \u03b1<jats:sup>0<\/jats:sup>\u2010thalassemia was identified in three subjects with Hb A<jats:sub>2<\/jats:sub>&gt;6.0%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Increased Hb A<jats:sub>2<\/jats:sub> level is a useful marker for differentiation of homozygous Hb E with and without \u03b1<jats:sup>0<\/jats:sup>\u2010thalassemia. This should lead to a significant reduction in number of referral cases of homozygous Hb E for molecular testing of \u03b1<jats:sup>0<\/jats:sup>\u2010thalassemia in routine practice.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijlh.12677","type":"journal-article","created":{"date-parts":[[2017,5,12]],"date-time":"2017-05-12T05:31:53Z","timestamp":1494567113000},"page":"508-512","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Co\u2010inheritance of \u03b1<sup>0<\/sup>\u2010thalassemia elevates Hb A<sub>2<\/sub> level in homozygous Hb E: Diagnostic implications"],"prefix":"10.1111","volume":"39","author":[{"given":"K.","family":"Singha","sequence":"first","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H.","family":"Srivorakun","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Fucharoen","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9142-5079","authenticated-orcid":false,"given":"S.","family":"Fucharoen","sequence":"additional","affiliation":[{"name":"Centre for Research and Development of Medical Diagnostic Laboratories Faculty of Associated Medical Sciences Khon Kaen University  Khon Kaen Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,5,12]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630268709036587"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-008-0490-z"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1159\/000438739"},{"key":"e_1_2_6_5_1","first-page":"1092","article-title":"Interaction of hemoglobin E and several forms of \u03b1\u2010thalassemia in Cambodian families","volume":"88","author":"Fucharoen S","year":"2003","journal-title":"Haematologica"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/9780470696705"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2008.09.002"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2013.11.006"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-003-0689-y"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-008-0520-x"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-006-0093-5"},{"key":"e_1_2_6_12_1","first-page":"364","article-title":"A simplified screening strategy for thalassaemia and haemoglobin E in rural communities in south\u2010east Asia","volume":"82","author":"Fucharoen G","year":"2004","journal-title":"Bull World Health Organ"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-553X.2009.01158.x"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1309\/LMMH649POETQREXL"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijlh.12585"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/aqw124"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1751-553X.2011.01301.x"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2011.09.008"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(94)90242-9"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2011.05.003"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinbiochem.2015.03.023"},{"key":"e_1_2_6_22_1","first-page":"2165","article-title":"Hemoglobin A2; 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