{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T03:53:03Z","timestamp":1791604383305,"version":"4.3.4"},"reference-count":19,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T00:00:00Z","timestamp":1791504000000},"content-version":"vor","delay-in-days":8,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000016","name":"U.S. Department of Health and Human Services","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000016","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["eJHaem"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>\n                      Red blood cell (RBC) deformability is a key determinant of sickle cell disease (SCD) pathophysiology. We evaluated RBC deformability across HbSS, HbAS, and HbAA genotypes and investigated its associations with hemolysis, fetal hemoglobin (HbF), coinherited \u03b1\u2010thalassemia, hydroxyurea treatment, and the\n                      <jats:italic>PIEZO1<\/jats:italic>\n                      E756del variant.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      We used Lorrca ektacytometry to compare RBC deformability across 174 participants with HbSS, HbAS, and HbAA genotypes. Deformability was evaluated across a range of shear stresses, osmotic pressures, and oxygen tensions. Associations between deformability and hemolysis markers, HbF levels, \u03b1\u2010thalassemia status, hydroxyurea use, and the\n                      <jats:italic>PIEZO1<\/jats:italic>\n                      E756del variant were assessed.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      HbSS RBCs showed markedly reduced deformability across all shear stresses, osmotic pressures, and oxygen tensions compared to HbAS and HbAA RBCs. Reduced deformability correlated with elevated markers of hemolysis, including lactate dehydrogenase (LDH) and bilirubin. Higher HbF levels were independently associated with improved RBC deformability, whereas coinherited \u03b1\u2010thalassemia was not associated with improved deformability. Hydroxyurea use was associated with significantly improved deformability. The\n                      <jats:italic>PIEZO1<\/jats:italic>\n                      E756del variant was associated with reduced deformability among participants with HbSS.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>RBC deformability is substantially impaired in HbSS and is associated with markers of hemolysis and several genetic and treatment\u2010related factors. These findings support Lorrca ektacytometry as a robust biomarker platform for assessing RBC health and treatment response in SCD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial Registration<\/jats:title>\n                    <jats:p>ClinicalTrials.gov identifier: NCT03685721<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/jha2.70416","type":"journal-article","created":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T03:29:19Z","timestamp":1791602959000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Probing Red Blood Cell Deformability in Sickle Cell Disease, Sickle Cell Trait, and Healthy Controls by Ektacytometry"],"prefix":"10.1002","volume":"7","author":[{"given":"Xunde","family":"Wang","sequence":"first","affiliation":[{"name":"Sickle Cell Branch National Heart, Lung, and Blood Institute National Institutes of Health (NIH)  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nancy","family":"Asomaning","sequence":"additional","affiliation":[{"name":"Sickle Cell Branch National Heart, Lung, and Blood Institute National Institutes of Health (NIH)  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haiou","family":"Li","sequence":"additional","affiliation":[{"name":"Sickle Cell Branch National Heart, Lung, and Blood Institute National Institutes of Health (NIH)  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen","family":"Lundt","sequence":"additional","affiliation":[{"name":"Sickle Cell Branch National Heart, Lung, and Blood Institute National Institutes of Health (NIH)  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Swee Lay","family":"Thein","sequence":"additional","affiliation":[{"name":"Sickle Cell Branch National Heart, Lung, and Blood Institute National Institutes of Health (NIH)  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,10,9]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2018.00656"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood\u20102017\u201002\u2010765891"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev\u2010bioeng\u2010070909\u2010105339"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev\u2010genom\u2010083117\u2010021320"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1177\/1535370219900494"},{"issue":"1","key":"e_1_2_10_7_1","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.bcmd.2015.09.001","article-title":"Diagnostic Tool for Red Blood Cell Membrane Disorders: Assessment of a New Generation Ektacytometer","volume":"56","author":"Da 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