{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T12:44:26Z","timestamp":1787921066316,"version":"build-2784847793"},"reference-count":18,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2018,2,14]],"date-time":"2018-02-14T00:00:00Z","timestamp":1518566400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000030","name":"Centers for Disease Control and Prevention","doi-asserted-by":"publisher","award":["71590"],"award-info":[{"award-number":["71590"]}],"id":[{"id":"10.13039\/100000030","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009741","name":"National Blood Foundation","doi-asserted-by":"publisher","award":["71590"],"award-info":[{"award-number":["71590"]}],"id":[{"id":"10.13039\/100009741","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["UL1 TR000424"],"award-info":[{"award-number":["UL1 TR000424"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2018,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Chronic transfusion therapy (CTT) for sickle cell disease (SCD) reduces disease morbidity by suppressing the amount of circulating hemoglobin S (HbS)\u2010containing red blood cells (RBC). The effectiveness of CTT depends on the rate of RBC clearance. Glucose\u20106\u2010phosphate dehydrogenase (G6PD) deficient donor RBC may exhibit increased hemolysis, but it is unknown if transfusion of these units results in less effective transfusion outcomes in SCD. Children with SCD on CTT were followed prospectively for multiple transfusions. G6PD activity of transfused units was measured prior to expiration date. HbA clearance (\u0394HbA) was calculated as the difference of estimated posttransfusion HbA to the pretransfusion HbA of the subsequent transfusion episode. Sixty\u2010two patients received 388 transfusions. Of 755 RBC units, 687 (91%) had normal G6PD (&gt;60% activity), 38 (5%) had moderately low G6PD (10\u201060% activity), and 30 (4%) had severely low G6PD (&lt;10% activity). Of 358 evaluable transfusions, 54 (15%) included \u22651 G6PD deficient units, and 22 (6%) had \u22651 severely deficient units. The proportion of the transfusion episode consisting of G6PD deficient units was associated with increased \u0394HbA for all G6PD deficient units (<jats:italic>P<\/jats:italic>\u2009=\u2009.05) and for severely G6PD deficient units (<jats:italic>P<\/jats:italic>\u2009=\u2009.0070). In multivariate mixed effects modeling, \u0394HbA was positively associated with severely G6PD deficient units (<jats:italic>P<\/jats:italic>\u2009=\u2009.0074) and RBC alloimmunization (<jats:italic>P<\/jats:italic>\u2009=\u2009.03) and negatively associated with recipient splenectomy (<jats:italic>P<\/jats:italic>\u2009=\u2009.015). Higher \u0394HbA was associated with higher HbS and reticulocyte counts at the subsequent transfusion episode. In conclusion, G6PD deficient RBC transfusions may have shorter in vivo survival and adversely affect the suppression of sickle erythropoiesis.<\/jats:p>","DOI":"10.1002\/ajh.25051","type":"journal-article","created":{"date-parts":[[2018,1,29]],"date-time":"2018-01-29T19:47:18Z","timestamp":1517255238000},"page":"630-634","source":"Crossref","is-referenced-by-count":44,"title":["Glucose\u20106\u2010phosphate\u2010dehydrogenase deficient red blood cell units are associated with decreased posttransfusion red blood cell survival in children with sickle cell disease"],"prefix":"10.1002","volume":"93","author":[{"given":"Eyal","family":"Sagiv","sequence":"first","affiliation":[{"name":"Division of Cardiology Children's Hospital Los Angeles, Los Angeles  California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ross M.","family":"Fasano","sequence":"additional","affiliation":[{"name":"Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine Emory University School of Medicine Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naomi L. C.","family":"Luban","sequence":"additional","affiliation":[{"name":"Center for Cancer and Blood Disorders, Children's National Medical Center, Departments of Hematology and Laboratory Medicine George Washington University School of Medicine and Health Sciences  Washington, D.C."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cassandra D.","family":"Josephson","sequence":"additional","affiliation":[{"name":"Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine Emory University School of Medicine Atlanta Georgia"},{"name":"Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics and Hematology\/Oncology Emory University School of Medicine Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean R.","family":"Stowell","sequence":"additional","affiliation":[{"name":"Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine Emory University School of Medicine Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John D.","family":"Roback","sequence":"additional","affiliation":[{"name":"Center for Transfusion and Cellular Therapies, Department of Pathology and Laboratory Medicine Emory University School of Medicine Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard O.","family":"Francis","sequence":"additional","affiliation":[{"name":"Department of Pathology and Cell Biology Columbia University Medical Center, New York Presbyterian Hospital New York New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6082-8385","authenticated-orcid":false,"given":"Marianne E. M.","family":"Yee","sequence":"additional","affiliation":[{"name":"Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics and Hematology\/Oncology Emory University School of Medicine Atlanta Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,2,14]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tmrv.2016.05.011"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/trf.14376"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOH.0b013e32833e5ac7"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2012.03765.x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/jca.21255"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcmd.2008.12.005"},{"key":"e_1_2_6_8_1","first-page":"601","article-title":"WHO Working Group","volume":"67","author":"Glucose\u20106\u2010phosphate dehydrogenase deficiency","year":"1989","journal-title":"Bull World Health Organ."},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.freeradbiomed.2016.04.005"},{"issue":"1","key":"e_1_2_6_10_1","first-page":"7","article-title":"Should blood donors be routinely screened for glucose\u20106\u2010phosphate dehydrogenase deficiency? A systematic review of clinical studies focusing on patients transfused with glucose\u20106\u2010phosphate dehydrogenase\u2010deficient red cells. 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