{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T09:09:00Z","timestamp":1789722540518,"version":"4.0.1"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T00:00:00Z","timestamp":1730246400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Haemoglobin (Hb) H disease and HbH disease with co\u2010inherited HbE mutation are the most prevalent forms of \u03b1\u2010thalassaemia in Southeast Asia. Data were limited when comparing clinical phenotypes between these two patient groups. We conducted a Thai multicentre study and enrolled 588 patients [median (IQR) age 13.0 (6.7\u201320.3) years], including those with deletional HbH disease with (<jats:italic>n<\/jats:italic>\u2009=\u200947) and without (<jats:italic>n<\/jats:italic>\u2009=\u2009187) co\u2010inherited HbE mutation and non\u2010deletional HbH disease with (<jats:italic>n<\/jats:italic>\u2009=\u2009101) and without (<jats:italic>n<\/jats:italic>\u2009=\u2009253) co\u2010inherited HbE mutation. Patients with HbH disease with co\u2010inherited HbE mutation suffered more severe manifestations than those without. This observation was more pronounced in patients with non\u2010deletional HbH disease. A greater proportion of patients with non\u2010deletional HbH disease with co\u2010inherited HbE mutation (43.6%) eventually required regular transfusions compared to those without (30.4%, <jats:italic>p<\/jats:italic>\u2009=\u20090.019). Among those with non\u2010deletional HbH disease who did not require regular transfusions, Hb levels were lower in patients with co\u2010inherited HbE mutation [8.1 (7.2\u20138.6) vs. 8.8 (8.2\u20139.5) g\/dL, <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001]. Among patients requiring regular transfusions who underwent splenectomy, 11\/12 patients with non\u2010deletional HbH disease stopped transfusion compared with 1\/3 in non\u2010deletional HbH disease with co\u2010inherited HbE mutation group (<jats:italic>p<\/jats:italic>\u2009=\u20090.024). These findings provide insights for the clinical monitoring and management of HbH disease in the region.<\/jats:p>","DOI":"10.1111\/bjh.19869","type":"journal-article","created":{"date-parts":[[2024,10,31]],"date-time":"2024-10-31T04:54:04Z","timestamp":1730350444000},"page":"703-712","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Impact of <scp>HbE<\/scp> mutation on the clinical severity of <scp>HbH<\/scp> disease: A\u00a0multicentre study from Thailand"],"prefix":"10.1111","volume":"206","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5750-7211","authenticated-orcid":false,"given":"Duantida","family":"Songdej","sequence":"first","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital Mahidol University  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2364-1498","authenticated-orcid":false,"given":"Nattiya","family":"Teawtrakul","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Srinagarind Hospital, Faculty of Medicine Khon Kaen University  Khon Kaen Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1517-9464","authenticated-orcid":false,"given":"Napat","family":"Laoaroon","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Khon Kaen University  Khon Kaen Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6804-9121","authenticated-orcid":false,"given":"Patcharee","family":"Komvilaisak","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Khon Kaen University  Khon Kaen Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5130-0714","authenticated-orcid":false,"given":"Pornpun","family":"Sripornsawan","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Prince of Songkla University  Songkhla Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4180-8041","authenticated-orcid":false,"given":"Pacharapan","family":"Surapolchai","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Thammasat University  Pathum Thani Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0996-3789","authenticated-orcid":false,"given":"Chattree","family":"Hantaweepant","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine, Faculty of Medicine Siriraj Hospital Mahidol University  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2381-9292","authenticated-orcid":false,"given":"Adisak","family":"Tantiworawit","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Chiang Mai University  Chiang Mai Thailand"},{"name":"Thalassemia and Hematology Center, Faculty of Medicine Chiang Mai University  Chiang Mai Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1248-9734","authenticated-orcid":false,"given":"Sasinee","family":"Hantrakool","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Chiang Mai University  Chiang Mai Thailand"},{"name":"Thalassemia and Hematology Center, Faculty of Medicine Chiang Mai University  Chiang Mai Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2432-8022","authenticated-orcid":false,"given":"Supanun","family":"Lauhasurayotin","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Chulalongkorn University and King Chulalongkorn Memorial Hospital  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kitti","family":"Torcharus","sequence":"additional","affiliation":[{"name":"Division of Pediatric Hematology\/Oncology, Department of Pediatrics Phramongkutklao College of Medicine  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pranee","family":"Sutcharitchan","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Chulalongkorn University and King Chulalongkorn Memorial Hospital  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4562-9139","authenticated-orcid":false,"given":"Noppacharn","family":"Uaprasert","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Chulalongkorn University and King Chulalongkorn Memorial Hospital  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krissana","family":"Panrong","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine Chulalongkorn University and King Chulalongkorn Memorial Hospital  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2679-4254","authenticated-orcid":false,"given":"Panachai","family":"Silpsamrit","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Phramongkutklao College of Medicine  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0336-0207","authenticated-orcid":false,"given":"Arunotai","family":"Meekaewkunchorn","sequence":"additional","affiliation":[{"name":"Queen Sirikit National Institute of Child Health  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9123-9177","authenticated-orcid":false,"given":"Pimlak","family":"Charoenkwan","sequence":"additional","affiliation":[{"name":"Thalassemia and Hematology Center, Faculty of Medicine Chiang Mai University  Chiang Mai Thailand"},{"name":"Division of Hematology and Oncology, Department of Pediatrics Chiang Mai University  Chiang Mai Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4204-0118","authenticated-orcid":false,"given":"Bunchoo","family":"Pongtanakul","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital Mahidol University  Bangkok Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"Red Blood Cell Disorders Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,10,30]]},"reference":[{"key":"e_1_2_12_2_1","first-page":"29","article-title":"Problems of thalassemia in Thailand","volume":"8","author":"Fucharoen S","year":"1988","journal-title":"ICMR Ann"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.40580"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-07-1975"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1010174"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/asheducation-2009.1.26"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.1081\/HEM-58583"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-009-0743-5"},{"issue":"9","key":"e_1_2_12_9_1","first-page":"908","article-title":"Genotypes and clinical features of 595 children with HbH disease in Guangxi, China","volume":"17","author":"He S","year":"2015","journal-title":"Zhongguo Dang Dai Er Ke Za Zhi"},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2022.12.004"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.21523"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.2001.03080.x"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1159\/000528510"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a011742"},{"key":"e_1_2_12_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2015.12.001"},{"key":"e_1_2_12_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2013.11.006"},{"key":"e_1_2_12_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.1988.tb00800.x"},{"key":"e_1_2_12_18_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a011734"},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.2147\/TACG.S161152"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1159\/000076523"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1159\/000435802"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbi.2019.103208"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbi.2008.08.010"},{"key":"e_1_2_12_24_1","volume-title":"Guidelines for the Management of non transfusion dependent thalassemia (NTDT)","author":"Taher AT","year":"2017"},{"key":"e_1_2_12_25_1","volume-title":"2021 guidelines for the management of transfusion dependent thalassemia (TDT)","author":"Cappellini MD","year":"2021"},{"key":"e_1_2_12_26_1","doi-asserted-by":"publisher","DOI":"10.1111\/ped.15568"},{"key":"e_1_2_12_27_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V96.7.2606"},{"key":"e_1_2_12_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02983382"},{"key":"e_1_2_12_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2006.06082.x"},{"key":"e_1_2_12_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s15010-011-0178-5"},{"key":"e_1_2_12_31_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2010.03940.x"},{"key":"e_1_2_12_32_1","doi-asserted-by":"publisher","DOI":"10.1159\/000347177"},{"key":"e_1_2_12_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1966.tb00125.x"},{"key":"e_1_2_12_34_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00277-014-2037-9"},{"key":"e_1_2_12_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1445-5994.1982.tb02655.x"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.19869","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,20]],"date-time":"2025-05-20T11:16:11Z","timestamp":1747739771000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.19869"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,30]]},"references-count":34,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2025,2]]}},"alternative-id":["10.1111\/bjh.19869"],"URL":"https:\/\/doi.org\/10.1111\/bjh.19869","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,30]]},"assertion":[{"value":"2024-06-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-10-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-10-30","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}