{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T22:06:50Z","timestamp":1794002810483,"version":"4.8.0"},"reference-count":54,"publisher":"American Society of Hematology","issue":"1","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,12,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>The transfusion of red blood cells (RBCs) is a crucial treatment for sickle cell disease (SCD). While often beneficial, the frequent use of transfusions is associated with numerous complications. Transfusions should be offered with specific guidelines in mind. Here we present updates to the indications for transfusion of RBCs in SCD. We review recent publications and include expert perspectives from hematology and transfusion medicine. For some clinical indications, such as ischemic stroke, the role of transfusion has been well studied and can be applied almost universally. For many other clinical scenarios, the use of transfusion therapy has less conclusive data and therefore must be tailored to individual needs. We highlight the roles of RBC transfusions in preventing or mitigating neurological disease, in reducing perioperative complications, in managing acute chest syndrome, and in optimizing pregnancy outcomes in SCD. We further highlight various transfusion techniques and when each might be considered. Potential complications of transfusion are also briefly discussed.<\/jats:p>","DOI":"10.1182\/hematology.2021000307","type":"journal-article","created":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T12:21:46Z","timestamp":1639138906000},"page":"696-703","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":54,"title":["Indications for transfusion in the management of sickle cell disease"],"prefix":"10.1182","volume":"2021","author":[{"given":"Hyojeong","family":"Han","sequence":"first","affiliation":[{"name":"Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Houston, TX"},{"name":"Department of Pediatrics, Baylor College of Medicine, Houston, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Hensch","sequence":"additional","affiliation":[{"name":"Division of Transfusion Medicine and Coagulation, Texas Children's Hospital, Houston, TX"},{"name":"Department of Pathology and Immunology and Anesthesiology, Baylor College of Medicine, Houston, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ven\u00e9e N.","family":"Tubman","sequence":"additional","affiliation":[{"name":"Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Houston, TX"},{"name":"Department of Pediatrics, Baylor College of Medicine, Houston, TX"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"234","published-online":{"date-parts":[[2021,12,10]]},"reference":[{"issue":"2","key":"2021121012092682300_hem2021000307C1","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.transci.2019.03.004","article-title":"Recommendations for the use of red blood cell exchange in sickle cell disease","volume":"58","author":"Fort","year":"2019","journal-title":"Transfus Apher Sci"},{"issue":"8","key":"2021121012092682300_hem2021000307C2","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1111\/trf.14806","article-title":"Red blood cell exchange in patients with sickle cell disease-indications and management: a review and consensus report by the therapeutic apheresis subsection of the AABB","volume":"58","author":"Biller","year":"2018","journal-title":"Transfusion"},{"issue":"2","key":"2021121012092682300_hem2021000307C3","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1097\/MPH.0000000000000645","article-title":"Transfusion therapy in children with sickle cell disease","volume":"39","author":"Inati","year":"2017","journal-title":"J Pediatr Hematol Oncol"},{"issue":"3","key":"2021121012092682300_hem2021000307C4","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1002\/jca.21705","article-title":"Guidelines on the use of therapeutic apheresis in clinical practice\u2014evidence-based approach from the Writing Committee of the American Society for Apheresis: the eighth special issue","volume":"34","author":"Padmanabhan","year":"2019","journal-title":"J Clin Apher"},{"issue":"4","key":"2021121012092682300_hem2021000307C5","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1111\/bjh.15115","article-title":"How I manage red cell transfusions in patients with sickle cell disease","volume":"180","author":"Rees","year":"2018","journal-title":"Br J Haematol"},{"issue":"6","key":"2021121012092682300_hem2021000307C6","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1067\/mpd.2001.119593","article-title":"Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia","volume":"139","author":"Miller","year":"2001","journal-title":"J Pediatr"},{"issue":"8","key":"2021121012092682300_hem2021000307C7","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1056\/NEJMoa1401731","article-title":"Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia","volume":"371","author":"DeBaun","year":"2014","journal-title":"N Engl J Med"},{"issue":"2","key":"2021121012092682300_hem2021000307C8","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1111\/bjh.14383","article-title":"Guidelines on red cell transfusion in sickle cell disease part II: indications for transfusion","volume":"176","author":"Davis","year":"2017","journal-title":"Br J Haematol"},{"issue":"1","key":"2021121012092682300_hem2021000307C9","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.cll.2020.10.007","article-title":"Transfusion and cellular therapy in pediatric sickle cell disease","volume":"41","author":"Zheng","year":"2021","journal-title":"Clin Lab Med"},{"issue":"1","key":"2021121012092682300_hem2021000307C10","doi-asserted-by":"crossref","first-page":"64","DOI":"10.3171\/2014.12.PEDS14522","article-title":"Encephaloduroarteriosynangiosis and encephalomyoarteriosynangiosis for treatment of moyamoya syndrome in pediatric patients with sickle cell disease","volume":"16","author":"Griessenauer","year":"2015","journal-title":"J Neurosurg Pediatr"},{"issue":"1","key":"2021121012092682300_hem2021000307C11","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: rates and risk factors","volume":"91","author":"Ohene-Frempong","year":"1998","journal-title":"Blood"},{"issue":"November","key":"2021121012092682300_hem2021000307C12","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1016\/j.cct.2017.08.008","article-title":"Design of the DREPAGREFFE trial: a prospective controlled multicenter study evaluating the benefit of genoidentical hematopoietic stem cell transplantation over chronic transfusion in sickle cell anemia children detected to be at risk of stroke by transcranial Doppler (NCT 01340404)","volume":"62","author":"Chevret","year":"2017","journal-title":"Contemp Clin Trials"},{"issue":"10019","key":"2021121012092682300_hem2021000307C13","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1016\/S0140-6736(15)01041-7","article-title":"Hydroxycarbamide versus chronic transfusion for maintenance of transcranial Doppler flow velocities in children with sickle cell anaemia-TCD With Transfusions Changing to Hydroxyurea (TWiTCH): a multicentre, open-label, phase 3, non-inferiority trial","volume":"387","author":"Ware","year":"2016","journal-title":"Lancet"},{"issue":"9","key":"2021121012092682300_hem2021000307C14","doi-asserted-by":"crossref","first-page":"E247","DOI":"10.1002\/ajh.25900","article-title":"Moderate fixed-dose hydroxyurea for primary prevention of strokes in Nigerian children with sickle cell disease: final results of the SPIN trial","volume":"95","author":"Galadanci","year":"2020","journal-title":"Am J Hematol"},{"issue":"8","key":"2021121012092682300_hem2021000307C15","doi-asserted-by":"crossref","first-page":"1554","DOI":"10.1182\/bloodadvances.2019001142","article-title":"American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults","volume":"4","author":"DeBaun","year":"2020","journal-title":"Blood Adv"},{"issue":"5","key":"2021121012092682300_hem2021000307C16","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1016\/j.jpeds.2006.06.037","article-title":"Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: a retrospective cohort study of 137 children with sickle cell anemia","volume":"149","author":"Hulbert","year":"2006","journal-title":"J Pediatr"},{"issue":"10","key":"2021121012092682300_hem2021000307C17","doi-asserted-by":"crossref","first-page":"1653","DOI":"10.1182\/blood-2014-09-599852","article-title":"Chronic and acute anemia and extracranial internal carotid stenosis are risk factors for silent cerebral infarcts in sickle cell anemia","volume":"125","author":"Bernaudin","year":"2015","journal-title":"Blood"},{"issue":"4","key":"2021121012092682300_hem2021000307C18","first-page":"CD012389","article-title":"Interventions for preventing silent cerebral infarcts in people with sickle cell disease","author":"Estcourt","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"issue":"October","key":"2021121012092682300_hem2021000307C19","doi-asserted-by":"crossref","first-page":"336","DOI":"10.1016\/j.jss.2019.04.077","article-title":"Acute chest syndrome after splenectomy in children with sickle cell disease","volume":"242","author":"El-Gohary","year":"2019","journal-title":"J Surg Res"},{"issue":"3","key":"2021121012092682300_hem2021000307C20","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.ijsu.2008.03.009","article-title":"Cholecystectomy in sickle cell disease patients: is there more acute chest syndrome after laparoscopy? A case controlled study","volume":"6","author":"Diarra","year":"2008","journal-title":"Int J Surg"},{"issue":"1","key":"2021121012092682300_hem2021000307C21","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1002\/ajh.25626","article-title":"Perioperative care of children with sickle cell disease: a systematic review and clinical recommendations","volume":"95","author":"Schyrr","year":"2020","journal-title":"Am J Hematol"},{"issue":"1","key":"2021121012092682300_hem2021000307C22","doi-asserted-by":"crossref","first-page":"e2018032","DOI":"10.4084\/mjhid.2018.032","article-title":"Perioperative management of sickle cell disease","volume":"10","author":"Adjepong","year":"2018","journal-title":"Mediterr J Hematol Infect Dis"},{"issue":"9870","key":"2021121012092682300_hem2021000307C23","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1016\/S0140-6736(12)61726-7","article-title":"The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: a randomised, controlled, multicentre clinical trial","volume":"381","author":"Howard","year":"2013","journal-title":"Lancet"},{"issue":"10","key":"2021121012092682300_hem2021000307C24","first-page":"CD008360","article-title":"Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease","author":"Estcourt","year":"2019","journal-title":"Cochrane Database Syst Rev"},{"issue":"2","key":"2021121012092682300_hem2021000307C25","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1182\/bloodadvances.2019001143","article-title":"American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support","volume":"4","author":"Chou","year":"2020","journal-title":"Blood Adv"},{"issue":"10","key":"2021121012092682300_hem2021000307C26","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1001\/jama.2014.10517","article-title":"Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members","volume":"312","author":"Yawn","year":"2014","journal-title":"JAMA"},{"issue":"2","key":"2021121012092682300_hem2021000307C27","first-page":"178","article-title":"Beneficial effect of blood transfusion in children with sickle cell chest syndrome","volume":"142","author":"Mallouh","year":"1988","journal-title":"Am J Dis Child"},{"issue":"25","key":"2021121012092682300_hem2021000307C28","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1056\/NEJM200006223422502","article-title":"Causes and outcomes of the acute chest syndrome in sickle cell disease","volume":"342","author":"Vichinsky","year":"2000","journal-title":"N Engl J Med"},{"issue":"3S","key":"2021121012092682300_hem2021000307C29","doi-asserted-by":"crossref","first-page":"S37","DOI":"10.1016\/j.annemergmed.2020.08.011","article-title":"Association of antibiotic choice with hospital length of stay and risk factors for readmission in patients with sickle cell disease and acute chest syndrome: an observational cohort study","volume":"76","author":"Badaki-Makun","year":"2020","journal-title":"Ann Emerg Med"},{"issue":"1","key":"2021121012092682300_hem2021000307C30","first-page":"CD007843","article-title":"Blood transfusions for treating acute chest syndrome in people with sickle cell disease","author":"Dolatkhah","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"issue":"8","key":"2021121012092682300_hem2021000307C31","first-page":"R","article-title":"Red blood cell transfusion to treat or prevent complications in sickle cell disease: an overview of Cochrane reviews","author":"Fortin","year":"2018","journal-title":"Cochrane Database Syst Rev"},{"issue":"7","key":"2021121012092682300_hem2021000307C32","doi-asserted-by":"crossref","first-page":"E154","DOI":"10.1002\/ajh.25809","article-title":"COVID-19 pneumonia as a cause of acute chest syndrome in an adult sickle cell patient","volume":"95","author":"Beerkens","year":"2020","journal-title":"Am J Hematol"},{"issue":"1","key":"2021121012092682300_hem2021000307C33","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1182\/bloodadvances.2020003456","article-title":"Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection","volume":"5","author":"Minniti","year":"2021","journal-title":"Blood Adv"},{"issue":"1","key":"2021121012092682300_hem2021000307C34","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1186\/s12890-021-01412-x","article-title":"Severe COVID-19 with acute respiratory distress syndrome (ARDS) in a sickle cell disease adult patient: case report","volume":"21","author":"Teulier","year":"2021","journal-title":"BMC Pulm Med"},{"issue":"4","key":"2021121012092682300_hem2021000307C35","doi-asserted-by":"crossref","first-page":"378","DOI":"10.1002\/jca.21809","article-title":"Red blood cell exchange to avoid intubating a COVID-19 positive patient with sickle cell disease?","volume":"35","author":"Allison","year":"2020","journal-title":"J Clin Apher"},{"issue":"18","key":"2021121012092682300_hem2021000307C36","doi-asserted-by":"crossref","first-page":"3431","DOI":"10.1182\/blood-2010-05-282194","article-title":"Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease","volume":"116","author":"Strouse","year":"2010","journal-title":"Blood"},{"issue":"August","key":"2021121012092682300_hem2021000307C37","first-page":"7080264","article-title":"Blood transfusion frequency and indications in Yemeni children with sickle cell disease","volume":"2020","author":"Al-Saqladi","year":"2020","journal-title":"Anemia"},{"issue":"June","key":"2021121012092682300_hem2021000307C38","first-page":"5253625","article-title":"Limited exchange transfusion can be very beneficial in sickle cell anemia with acute chest syndrome: a case report from Tanzania","volume":"2018","author":"Chamba","year":"2018","journal-title":"Case Rep Hematol"},{"issue":"8","key":"2021121012092682300_hem2021000307C39","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1111\/trf.15818","article-title":"Blood utilization and characteristics of patients treated with chronic transfusion therapy in a large cohort of Brazilian patients with sickle cell disease","volume":"60","author":"Kelly","year":"2020","journal-title":"Transfusion"},{"issue":"3","key":"2021121012092682300_hem2021000307C40","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1080\/21548331.2018.1464363","article-title":"Acute chest syndrome in sickle cell disease","volume":"46","author":"Farooq","year":"2018","journal-title":"Hosp Pract (1995)"},{"issue":"1","key":"2021121012092682300_hem2021000307C41","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1182\/asheducation-2014.1.418","article-title":"Reproductive issues in sickle cell disease","volume":"2014","author":"Smith-Whitley","year":"2014","journal-title":"Hematology Am Soc Hematol Educ Program"},{"issue":"9","key":"2021121012092682300_hem2021000307C42","doi-asserted-by":"crossref","first-page":"872","DOI":"10.1002\/ajh.24790","article-title":"Implementation of multidisciplinary care reduces maternal mortality in women with sickle cell disease living in low-resource setting","volume":"92","author":"Asare","year":"2017","journal-title":"Am J Hematol"},{"issue":"21","key":"2021121012092682300_hem2021000307C43","doi-asserted-by":"crossref","first-page":"2424","DOI":"10.1182\/blood-2015-06-649319","article-title":"Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis","volume":"126","author":"Malinowski","year":"2015","journal-title":"Blood"},{"issue":"9","key":"2021121012092682300_hem2021000307C44","doi-asserted-by":"crossref","first-page":"2192","DOI":"10.1111\/trf.14767","article-title":"Improvement of maternal and fetal outcomes in women with sickle cell disease treated with early prophylactic erythrocytapheresis","volume":"58","author":"Vianello","year":"2018","journal-title":"Transfusion"},{"issue":"1","key":"2021121012092682300_hem2021000307C45","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1186\/s13063-020-4212-8","article-title":"Serial prophylactic exchange blood transfusion in pregnant women with sickle cell disease (TAPS-2): study protocol for a randomised controlled feasibility trial","volume":"21","author":"Oakley","year":"2020","journal-title":"Trials"},{"issue":"3","key":"2021121012092682300_hem2021000307C46","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1016\/j.tracli.2015.05.005","article-title":"Mechanisms of sickle cell alloimmunization","volume":"22","author":"Yazdanbakhsh","year":"2015","journal-title":"Transfus Clin Biol"},{"issue":"12","key":"2021121012092682300_hem2021000307C47","doi-asserted-by":"crossref","first-page":"1135","DOI":"10.1002\/ajh.24188","article-title":"Immunophenotypic parameters and RBC alloimmunization in children with sickle cell disease on chronic transfusion","volume":"90","author":"Nickel","year":"2015","journal-title":"Am J Hematol"},{"issue":"6","key":"2021121012092682300_hem2021000307C48","doi-asserted-by":"crossref","first-page":"488","DOI":"10.1097\/MOH.0000000000000546","article-title":"The cause and pathogenesis of hemolytic transfusion reactions in sickle-cell disease","volume":"26","author":"Pirenne","year":"2019","journal-title":"Curr Opin Hematol"},{"issue":"7","key":"2021121012092682300_hem2021000307C49","doi-asserted-by":"crossref","first-page":"1707","DOI":"10.1111\/trf.13558","article-title":"Effectiveness of red blood cell exchange, partial manual exchange, and simple transfusion concurrently with iron chelation therapy in reducing iron overload in chronically transfused sickle cell anemia patients","volume":"56","author":"Fasano","year":"2016","journal-title":"Transfusion"},{"issue":"5","key":"2021121012092682300_hem2021000307C50","first-page":"258","article-title":"A phase I\/II study of polymerized bovine hemoglobin in adult patients with sickle cell disease not in crisis at the time of study","volume":"45","author":"Gonzalez","year":"1997","journal-title":"J Investig Med"},{"issue":"1","key":"2021121012092682300_hem2021000307C51","doi-asserted-by":"crossref","first-page":"2","DOI":"10.3390\/biom7010002","article-title":"Hemoglobin-based blood substitutes and the treatment of sickle cell disease: more harm than help?","volume":"7","author":"Alayash","year":"2017","journal-title":"Biomolecules"},{"issue":"1","key":"2021121012092682300_hem2021000307C52","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1111\/trf.14386","article-title":"Use of the blood substitute HBOC-201 in critically ill patients during sickle crisis: a three-case series","volume":"58","author":"Davis","year":"2018","journal-title":"Transfusion"},{"issue":"5","key":"2021121012092682300_hem2021000307C53","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1111\/jcpt.12998","article-title":"Successful management of the potentially fatal hyperhaemolysis syndrome of sickle cell anaemia with a regimen including bortezomib and Hemopure","volume":"44","author":"Epstein","year":"2019","journal-title":"J Clin Pharm Ther"},{"issue":"6","key":"2021121012092682300_hem2021000307C54","doi-asserted-by":"crossref","first-page":"1907","DOI":"10.1111\/trf.15198","article-title":"Anti-N and anti-Doa immunoglobulin G alloantibody-mediated delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease treated with eculizumab and HBOC-201: case report and review of the literature","volume":"59","author":"Unnikrishnan","year":"2019","journal-title":"Transfusion"}],"container-title":["Hematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2021\/1\/696\/1851821\/696han.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/ashpublications.org\/hematology\/article-pdf\/2021\/1\/696\/1851821\/696han.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,12,10]],"date-time":"2021-12-10T12:22:42Z","timestamp":1639138962000},"score":1,"resource":{"primary":{"URL":"https:\/\/ashpublications.org\/hematology\/article\/2021\/1\/696\/482990\/Indications-for-transfusion-in-the-management-of"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12,10]]},"references-count":54,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2021,12,10]]},"published-print":{"date-parts":[[2021,12,10]]}},"URL":"https:\/\/doi.org\/10.1182\/hematology.2021000307","relation":{},"ISSN":["1520-4391","1520-4383"],"issn-type":[{"value":"1520-4391","type":"print"},{"value":"1520-4383","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,12,10]]},"published":{"date-parts":[[2021,12,10]]}}}