Abstract：
Crizanlizumab, a monoclonal antibody against P-selectin, has been shown to reduce vaso-occlusive crises (VOCs) compared to placebo in patients ≥ 16 years with sickle cell disease (SCD). However, there have been rare reports of patients experiencing severe pain and subsequent complications within 24 hours of crizanlizumab infusions. These events are defined as infusion-related reactions (IRRs). Informed by current literature and clinical experience, a group of content experts developed clinical guidelines for the management of IRRs in patients with SCD. We used the RAND/University of California, Los Angeles (UCLA) modified Delphi panel method, a valid, reproducible technique for achieving consensus. We present our recommendations for managing IRRs, which depend on patient characteristics including: prior history of IRRs to other monoclonal antibodies or medications, changes to crizanlizumab infusion rate and patient monitoring, pain severity relative to patient\'s typical SCD crises, and severe allergic symptoms. These recommendations outline how to evaluate and manage IRRs in patients receiving crizanlizumab. Future research should validate this guidance using clinical data and identify patients at risk for these IRRs.
摘要：
Crizanlizumab,一种抗P-选择素的单克隆抗体,在≥16岁的镰状细胞病（SCD）患者中，与安慰剂相比，已证明可减少血管闭塞危象（VOCs）。然而,很少有报道称患者在接受crizanlizumab输注后24小时内出现严重疼痛和随后的并发症.这些事件定义为输注相关反应(IRR)。根据现有文献和临床经验，一组内容专家制定了SCD患者IRRs管理的临床指南.我们使用兰德大学/加州大学，洛杉矶(加州大学洛杉矶分校)改进的德尔菲面板法，一个有效的，实现共识的可重复技术。我们提出了管理内部收益率的建议，取决于患者特征，包括：其他单克隆抗体或药物的IRR的既往史，crizanlizumab输注速率和患者监测的变化，疼痛严重程度相对于患者的典型SCD危机，和严重的过敏症状。这些建议概述了如何评估和管理接受crizanlizumab的患者的IRR。未来的研究应该使用临床数据验证这一指导，并确定有这些IRR风险的患者。
