{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T11:39:46Z","timestamp":1790509186597,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2018,11,25]],"date-time":"2018-11-25T00:00:00Z","timestamp":1543104000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008272","name":"Novartis Pharmaceuticals Corporation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J Hematol"],"published-print":{"date-parts":[[2019,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>The cell adhesion molecule P\u2010selectin plays a key role in the pathogenesis of a vaso\u2010occlusive crisis (VOC) in patients with sickle cell disease (SCD). In the double\u2010blind, placebo\u2010controlled phase 2 SUSTAIN study, crizanlizumab (humanized, anti\u2010P\u2010selectin monoclonal antibody) 5 mg\/kg significantly lowered the rate of VOC in patients with SCD by 45% vs placebo. In SUSTAIN, patients with SCD were randomized to crizanlizumab 2.5 mg\/kg, crizanlizumab 5\u2009mg\/kg, or placebo intravenously 14 times over 52 weeks. The primary endpoint was the annual rate of VOC with crizanlizumab vs placebo. This post hoc descriptive analysis evaluated the proportion of patients who did not experience a VOC during the study in the following subgroups: VOCs in the year prior to study entry (2\u20104\/5\u201010), SCD genotype (HbSS\/non\u2010HbSS), and concomitant hydroxyurea use (yes\/no). More patients were VOC event\u2010free in the crizanlizumab 5 mg\/kg arm than in the placebo arm, including those with more frequent prior VOCs (ie, 5\u201010; 28.0% vs 4.2%), the HbSS genotype (31.9% vs 17.0%) and\/or using concomitant hydroxyurea (33.3% vs 17.5%). Further analyses of secondary endpoints demonstrated that crizanlizumab treatment significantly increased time\u2010to\u2010first VOC vs placebo in these subgroups. The rates of treatment\u2010emergent adverse events were similar between treatment arms across all subgroups. This post hoc analysis of SUSTAIN shows that in patients with a high number of prior VOCs, on concomitant hydroxyurea and\/or with the HbSS genotype, crizanlizumab treatment increases the likelihood of patients being VOC event\u2010free and delays time\u2010to\u2010first VOC.<\/jats:p>","DOI":"10.1002\/ajh.25308","type":"journal-article","created":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T06:54:56Z","timestamp":1538981696000},"page":"55-61","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":113,"title":["Effect of crizanlizumab on pain crises in subgroups of patients with sickle cell disease: A SUSTAIN study analysis"],"prefix":"10.1002","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5482-8087","authenticated-orcid":false,"given":"Abdullah","family":"Kutlar","sequence":"first","affiliation":[{"name":"Sickle Cell Center Medical College of Georgia, Augusta University  Augusta 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