{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T11:31:54Z","timestamp":1792063914847,"version":"4.4.0"},"reference-count":29,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2019,1,29]],"date-time":"2019-01-29T00:00:00Z","timestamp":1548720000000},"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":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2019,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Regular blood transfusion therapy still remains the cornerstone in the management of \u03b2\u2010thalassemia. Although recommendations are clear for patients with \u03b2\u2010thalassemia major, uniform transfusion guidelines are lacking for patients with hemoglobin E \u03b2\u2010thalassemia. In this study, we aim to describe the adequacy, trends, and determinants of blood transfusion therapy in a large cohort of pediatric patients with \u03b2\u2010thalassemia major and hemoglobin E \u03b2\u2010thalassemia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods\/procedure<\/jats:title><jats:p>This cross\u2010sectional study was performed among all regularly transfused patents with \u03b2\u2010thalassemia aged 2 to 18 years attending three large thalassemia centers in Sri Lanka. Data were collected using an interviewer\u2010administered questionnaire, perusal of clinical records, and physical examination of patients by trained doctors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 328 patients (male 47%) were recruited; 83% had \u03b2\u2010thalassemia major, whereas 16% had hemoglobin E \u03b2\u2010thalassemia. Sixty\u2010one percent of patients had low pretransfusion hemoglobin levels (&lt;\u00a09.0\u00a0g\/dL) despite receiving high transfusion volumes (&gt;\u00a0200\u00a0mL\/kg\/year) by a majority (56%). Median pretransfusion hemoglobin was significantly lower in patients with hemoglobin E \u03b2\u2010thalassemia compared with \u03b2\u2010thalassemia major (<jats:italic>P<\/jats:italic>\u00a0&lt;\u00a00.001); however, there was no difference in requirement for high transfusion volumes over 200\u00a0mL\/kg\/year in both groups (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.14). Hepatomegaly and splenomegaly were more common in hemoglobin E \u03b2\u2010thalassemia and were associated with lower pretransfusion hemoglobin. Transfusion requirements were higher among patients with hepatitis C and in those who are underweight.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Over 60% of regularly transfused patients with \u03b2\u2010thalassemia have low pretransfusion hemoglobin levels despite receiving large transfusion volumes. Patients with hemoglobin E \u03b2\u2010thalassemia are undertransfused and specific recommendations should be developed to guide transfusions in these patients.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.27643","type":"journal-article","created":{"date-parts":[[2019,1,30]],"date-time":"2019-01-30T05:31:15Z","timestamp":1548826275000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Blood transfusion therapy for \u03b2\u2010thalassemia major and hemoglobin E \u03b2\u2010thalassemia: Adequacy, trends, and determinants in Sri Lanka"],"prefix":"10.1002","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0760-0418","authenticated-orcid":false,"given":"Sachith","family":"Mettananda","sequence":"first","affiliation":[{"name":"Department of Paediatrics Faculty of Medicine University of Kelaniya  Sri Lanka"},{"name":"Colombo North Teaching Hospital  Ragama Sri 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Silva","sequence":"additional","affiliation":[{"name":"Anuradhapura Teaching Hospital  Anuradhapura Sri Lanka"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chamila","family":"Mettananda","sequence":"additional","affiliation":[{"name":"Department of Pharmacology Faculty of Medicine University of Kelaniya  Sri Lanka"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anuja","family":"Premawardhena","sequence":"additional","affiliation":[{"name":"Colombo North Teaching Hospital  Ragama Sri Lanka"},{"name":"Department of Medicine Faculty of Medicine University of Kelaniya  Sri 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