{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T14:59:54Z","timestamp":1787237994520,"version":"build-2736575974"},"reference-count":23,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,2,15]],"date-time":"2021-02-15T00:00:00Z","timestamp":1613347200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Red cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0.027\/100 units of red cells. The absence of any iron loading was confirmed by serial Ferriscans, performed over a number of years. However, patients with advanced chronic kidney disease showed evidence of iron loading due to reduced innate haemopoiesis and were subsequently switched to simple transfusions. A total of 59% of patients were on regular automated red cell exchange with a history of recurrent painful crises. A total of 77% responded clinically, as evidenced by at least a 25% reduction in their emergency hospital attendance for pain management. The clinical response was gradual and increased the longer patients stayed on the program. The earliest sign of clinical response was a reduction in the length of stay when these patients were hospitalised, indicating that a reduction in the severity of crises precedes the reduction in their frequency. Automated red cell exchange also appeared to be beneficial for patients with recurrent leg ulcers and severe, drug resistant stuttering priapism, while patients with pulmonary hypertension showed a dramatic improvement in their symptoms as well as echocardiographic parameters.<\/jats:p>","DOI":"10.3390\/jcm10040767","type":"journal-article","created":{"date-parts":[[2021,2,15]],"date-time":"2021-02-15T03:22:26Z","timestamp":1613359346000},"page":"767","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":29,"title":["Automated Red Cell Exchange in the Management of Sickle Cell Disease"],"prefix":"10.3390","volume":"10","author":[{"given":"Dimitris A.","family":"Tsitsikas","sequence":"first","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saket","family":"Badle","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhys","family":"Hall","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Meenan","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oloruntoyin","family":"Bello-Sanyaolu","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Funmilayo","family":"Orebayo","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jibril","family":"Abukar","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Elmi","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Afsana","family":"Mulla","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shalini","family":"Dave","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha","family":"Lewis","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manisha","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Basabi","family":"Chatterjee","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger J.","family":"Amos","sequence":"additional","affiliation":[{"name":"Haemoglobinopathy Service, Department of Haematology, Homerton University Hospital NHS Foundation Trust, London E9 6SR, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,15]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1111\/bjh.14383","article-title":"British Society for Haematology. 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."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1002\/jca.21705","article-title":"Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-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."},{"key":"ref_3","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."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1053\/j.ajkd.2018.12.027","article-title":"Progressive Decline in Estimated GFR in Patients with Sickle Cell Disease: An Observational Cohort Study","volume":"74","author":"Derebail","year":"2019","journal-title":"Am. J. Kidney Dis."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1111\/bjh.14346","article-title":"British Committee for Standards in Haematology. Guidelines on red cell transfusion in sickle cell disease. Part I: Principles and laboratory aspects","volume":"176","author":"Davis","year":"2017","journal-title":"Br. J. Haematol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1002\/jca.21447","article-title":"Safety, tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease","volume":"31","author":"Tsitsikas","year":"2016","journal-title":"J. Clin. Apher."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1182\/blood-2013-03-490623","article-title":"High prevalence of red blood cell alloimmunization in sickle cell disease despite transfusion from Rh-matched minority donors","volume":"122","author":"Chou","year":"2013","journal-title":"Blood"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e212","DOI":"10.1111\/bjh.16103","article-title":"Treatment of post-transfusion hyperhaemolysis syndrome in Sickle Cell Disease with the anti-IL6R humanised monoclonal antibody Tocilizumab","volume":"186","author":"Sivapalaratnam","year":"2019","journal-title":"Br. J. Haematol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2671","DOI":"10.1111\/j.1537-2995.2012.03659.x","article-title":"Lower alloimmunization rates in pediatric sickle cell patients on chronic erythrocytapheresis compared to chronic simple transfusions","volume":"52","author":"Wahl","year":"2012","journal-title":"Transfusion"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1111\/trf.12875","article-title":"Immunohematologic tolerance of chronic transfusion exchanges with erythrocytapheresis in sickle cell disease","volume":"55","author":"Michot","year":"2015","journal-title":"Transfusion"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"de Montalembert, M., Ribeil, J.A., Brousse, V., Guerci-Bresler, A., Stamatoullas, A., Vannier, J.P., Dumesnil, C., Lahary, A., Touati, M., and Bouabdallah, K. (2017). Cardiac iron overload in chronically transfused patients with thalassemia, sickle cell anemia, or myelodysplastic syndrome. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0172147"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/j.transci.2017.08.001","article-title":"Distinct patterns of response to transfusion therapy for different chronic complications of sickle cell disease: A useful insight","volume":"56","author":"Tsitsikas","year":"2017","journal-title":"Transfus. Apher. Sci."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1111\/bjh.13031","article-title":"Regular automated red cell exchange transfusion in the management of pulmonary hypertension in sickle cell disease","volume":"167","author":"Tsitsikas","year":"2014","journal-title":"Br. J. Haematol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1182\/blood-2015-08-618561","article-title":"Pathophysiology and treatment of pulmonary hypertension in sickle cell disease","volume":"127","author":"Gordeuk","year":"2016","journal-title":"Blood"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1056\/NEJMoa035477","article-title":"Pulmonary hypertension as a risk factor for death in patients with sickle cell disease","volume":"350","author":"Gladwin","year":"2004","journal-title":"N. Engl. J. Med."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3867","DOI":"10.1182\/bloodadvances.2019000916","article-title":"American Society of Hematology 2019 guidelines for sickle cell disease: Cardiopulmonary and kidney disease","volume":"3","author":"Liem","year":"2019","journal-title":"Blood Adv."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1111\/bjh.14393","article-title":"Regular automated red cell exchange transfusion in the management of stuttering priapism complicating sickle cell disease","volume":"180","author":"Ekong","year":"2018","journal-title":"Br. J. Haematol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1056\/NEJMoa1903212","article-title":"HOPE Trial Investigators. A Phase 3 Randomized Trial of Voxelotor in Sickle Cell Disease","volume":"381","author":"Vichinsky","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1016\/j.transci.2017.05.020","article-title":"A 5-year cost analysis of automated red cell exchange transfusion for the management of recurrent painful crises in adult patients with sickle cell disease","volume":"56","author":"Tsitsikas","year":"2017","journal-title":"Transfus. Apher. Sci."},{"key":"ref_20","first-page":"836","article-title":"Indications for RBC Exchange Transfusion in Patients with Sickle Cell Disease: Revisited","volume":"49","author":"Ballas","year":"2019","journal-title":"Ann. Clin. Lab. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1177\/0885066619874041","article-title":"Reversal of Severe Multiorgan Failure Associated With Sickle Cell Crisis Using Plasma Exchange: A Case Series","volume":"35","author":"Zaidi","year":"2020","journal-title":"J. Intensive Care Med."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"E629","DOI":"10.1002\/ajh.24870","article-title":"Plasmapheresis may improve clinical condition in sickle cell disease through its effects on red blood cell rheology","volume":"92","author":"Nader","year":"2017","journal-title":"Am. J. Hematol."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Tsitsikas, D.A., Vize, J., and Abukar, J. (2020). Fat Embolism Syndrome in Sickle Cell Disease. J. Clin. Med., 9.","DOI":"10.3390\/jcm9113601"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/4\/767\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:24:17Z","timestamp":1760160257000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/4\/767"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,15]]},"references-count":23,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2021,2]]}},"alternative-id":["jcm10040767"],"URL":"https:\/\/doi.org\/10.3390\/jcm10040767","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,15]]}}}