{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T08:10:23Z","timestamp":1780387823379,"version":"3.54.1"},"reference-count":34,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T00:00:00Z","timestamp":1776816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T00:00:00Z","timestamp":1776816000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Anemia results from imbalanced hemoglobin or red blood cell production and clearance. Hemolytic anemia, caused by premature red blood cell removal, can be intravascular (in blood) or extravascular (erythrophagocytosis). Hemolysis is common in Sickle Cell Disease (SCD) and Beta\u2010Thalassemia anemia (\u03b2\u2010thalassemia), the most prevalent inherited hemolytic anemias. Hemolysis severity is primarily assessed by measuring indirect serum biomarkers such as lactate dehydrogenase, released by cytolysis, bilirubin and haptoglobin. However, these markers do not directly indicate either the cause or the primary site of hemolysis. We introduced a novel plasma heme assay that quantifies all heme\u2010related species in plasma, including hemoglobin, methemoglobin, heme, and hemopexin. Our findings revealed a more profound intravascular red blood cell destruction in SCD compared to \u03b2\u2010thalassemia as demonstrated by higher values of plasma hemoglobin, respectively 6.20 and 2.52\u2009\u03bcM (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001), with significant inter\u2010individual variability. In contrast, \u03b2\u2010thalassemia patients exhibited higher plasma heme values (11.00\u2009\u03bcM vs. 1.51\u2009\u03bcM;\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.0001) reflecting a probable mixed origin (dyserythropoiesis and hemolysis). Plasma hemopexin was negatively correlated with plasma heme in all patients. Plasma heme exceeded hemopexin scavenging capability in 72% of \u03b2\u2010thalassemia and 36% of SCD patients. In \u03b2\u2010thalassemia, plasma heme levels were significantly higher in transfusion\u2010dependent compared to non\u2013transfusion\u2010dependent patients, indicating that excess heme reflects clinical severity. In SCD, elevated concentration of excess heme was associated with a significant increased risk of mortality compared to LDH or reticulocytes%. This novel spectral assay offered significant benefits for diagnosis, treatment, and patient management.\n                  <\/jats:p>","DOI":"10.1002\/ajh.70314","type":"journal-article","created":{"date-parts":[[2026,4,23]],"date-time":"2026-04-23T04:00:32Z","timestamp":1776916832000},"page":"1706-1716","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Novel Plasma Heme Assay Reveals Disease Severity in Beta\u2010Thalassemia and Sickle Cell Anemia"],"prefix":"10.1002","volume":"101","author":[{"given":"Laurent","family":"Kiger","sequence":"first","affiliation":[{"name":"Institut Mondor de Recherche Biom\u00e9dicale  Cr\u00e9teil France"},{"name":"Sickle Cell Referral Center, UMGGR, Plateforme D\u2019expertise Maladies Rares Grand Paris Est UPEC H\u00f4pitaux Universitaires Henri Mondor, APHP  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0232-831X","authenticated-orcid":false,"given":"Nicolas","family":"Hebert","sequence":"additional","affiliation":[{"name":"Institut Mondor de Recherche Biom\u00e9dicale  Cr\u00e9teil France"},{"name":"Sickle Cell Referral Center, UMGGR, Plateforme D\u2019expertise Maladies Rares Grand Paris Est UPEC H\u00f4pitaux Universitaires Henri Mondor, APHP  Cr\u00e9teil France"},{"name":"Etablissement Fran\u00e7ais du Sang  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Bencheikh","sequence":"additional","affiliation":[{"name":"Institut Mondor de Recherche Biom\u00e9dicale  Cr\u00e9teil France"},{"name":"INNOVHEM  Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent Malcor Deydier","family":"de Pierrefeu","sequence":"additional","affiliation":[{"name":"Institut Mondor de Recherche Biom\u00e9dicale  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