{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T21:00:55Z","timestamp":1791320455132,"version":"4.3.1"},"reference-count":28,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2016,7,14]],"date-time":"2016-07-14T00:00:00Z","timestamp":1468454400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2016,10]]},"abstract":"<jats:p>Delayed hemolytic transfusion reaction (DHTR) is one of the most feared complications of sickle\u2010cell disease (SCD). We retrospectively analyzed the clinical and biological features, treatments and outcomes of 99 DHTRs occurring in 69 referral center patients over 12 years. The first clinical signs appeared a median of 9.4 [IQR, 3\u201322] days after the triggering transfusion (TT). The most frequent DHTR\u2010related clinical manifestation was dark urine\/hemoglobinuria (94%). Most patients (89%) had a painful vaso\u2010occlusive crisis and 50% developed a secondary acute chest syndrome (ACS). The median [IQR] hemoglobin\u2010concentration nadir was 5.5 [4.5\u20136.3] g\/dL and LDH peak was 1335 [798\u20132086] IU\/L. Overall mortality was 6%. None of the patients had been receiving chronic transfusions. Among these DHTRs, 61% were developed in previously immunized patients, 28% in patients with prior DHTR. Among Abs detected after the TT in 62% of the episodes, half are classically considered potentially harmful. No association could be established between clinical severity and immunohematological profile and\/or the type and specificity of Abs detected after the TT. Management consisted of supportive care alone (53%) or with adjunctive measures (47%), including recombinant erythropoietin and sometimes rituximab and\/or immunosuppressants. Additional transfusions were either ineffective or worsened hemolysis. In some cases, severe intravascular hemolysis can be likely responsible for the vascular reaction and high rates of ACS, pulmonary hypertension and (multi)organ failure. In conclusion, clinicians and patients must recognize early DHTR signs to avoid additional transfusions. For patients with a history of RBC immunization or DHTR, transfusion indications should be restricted. Am. J. Hematol. 91:989\u2013994, 2016. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.24460","type":"journal-article","created":{"date-parts":[[2016,6,27]],"date-time":"2016-06-27T22:52:46Z","timestamp":1467067966000},"page":"989-994","source":"Crossref","is-referenced-by-count":119,"title":["Delayed hemolytic transfusion reaction in adult sickle\u2010cell disease: presentations, outcomes, and treatments of 99 referral center episodes"],"prefix":"10.1002","volume":"91","author":[{"given":"Anoosha","family":"Habibi","sequence":"first","affiliation":[{"name":"Unit\u00e9 des Maladies G\u00e9n\u00e9tiques du Globule Rouge, APHP, H\u00f4pitaux Universitaire Henri\u2010Mondor Cr\u00e9teil France"},{"name":"UPEC, Institut Mondor de Recherche Biom\u00e9dicale (IMRB), Institut National de la Sant\u00e9 et de le Recherche M\u00e9dicale (INSERM) U955 DHU A\u2010TVB Cr\u00e9teil 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