ADAMTS13号
血栓性血小板减少性紫癜
医学
内科学
危险系数
胃肠病学
比例危险模型
心脏病学
置信区间
血小板
作者
Laure De Waele,Kazuya Sakai,Ilaria Mancini,György Sinkovits,Tanja Falter,Takashi Inoue,Pasquale Agosti,Heidi Rossmann,Charis von Auer,Claudia Tersteeg,Simon F. De Meyer,Bérangère S. Joly,Agnès Veyradier,Paul Coppo,Rob Fijnheer,Flora Peyvandi,Zoltán Prohászka,Bernhard Lämmle,Karen Vanhoorelbeke
标识
DOI:10.1016/j.jtha.2023.10.014
摘要
Background ADAMTS13 adopts an open conformation in immune-mediated thrombotic thrombocytopenic purpura (iTTP) patients in acute phase while being closed in healthy donors. We reported that a substantial number of iTTP patients in remission with restored ADAMTS13 activity (>50%) still had an open ADAMTS13 conformation, although a closed conformation is expected given the extent of remission. Objective To investigate whether open ADAMTS13, represented by conformation index >0.5, is associated with a risk for earlier ADAMTS13 and/or clinical relapse. Patients/Methods We collected follow-up data (ADAMTS13 parameters, ADAMTS13 and clinical relapse and treatment) from 81 iTTP patients in remission with ADAMTS13 activity above 50%. Results During follow-up, 19 ADAMTS13 and 10 clinical relapses were reported (median follow up period: 20 months). First, open or closed ADAMTS13 conformation was dichotomized based on the 0.5 conformation index cut-off. Open ADAMTS13 (conformation index >0.5) was not identified as a risk for ADAMTS13 and clinical relapse (log-rank test and Cox regression model). In contrast, by identifying the optimal conformation index cut-off for relapse prediction, using classification and regression tree analysis, conformation index >0.645 and >0.835 were shown to be a risk for respectively ADAMTS13 relapse (hazard ratio (HR) 3.3, (95% CI:1.3-8.3), p=0.01) and clinical relapse (HR 4.4, (95% CI:1.3-15.3), p=0.02). Conclusion Patients with open ADAMTS13 with conformation index >0.645 and >0.835, respectively have an over 3- and 4-fold higher risk for earlier ADAMTS13 and clinical relapse. Hence, ADAMTS13 conformation index could be used to complement ADAMTS13 activity monitoring to timely notice ADAMTS13 relapse and prevent clinical relapse.
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