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在镰状细胞病的慢性管理中,进行自动化红细胞置换,术后血细胞比容目标设定为34%。

Automated red blood cell exchange with a post-procedure haematocrit targeted at 34% in the chronic management of sickle cell disease.

作者信息

M Ross Jules, Forté Stéphanie, Mercure-Corriveau Nicolas, Lemay Anne-Sophie, Rioux-Massé Benjamin, Potter Brian J, Soulières Denis

机构信息

Hematology-Oncology Division, Departement of Medicine, Centre Hospitalier de l'Université de Montréal, Montreal, Canada.

Cardiology Division, Department of Medicine, Centre Hospitalier de l'Université de Montréal, Montreal, Canada.

出版信息

Br J Haematol. 2024 Oct;205(4):1556-1564. doi: 10.1111/bjh.19674. Epub 2024 Jul 30.

Abstract

Optimal targets for red blood cell exchange (RCE) are not well defined in the chronic management of sickle cell disease. We analysed transfusion requirements and iron-related outcomes in 101 patients on chronic RCE with a post-procedure haematocrit (Ht) targeted at 34%, which is higher than typically used. A majority were of HbSS/HbSβ0 genotype (n = 72) and enrolled for neurological complications (n = 53). Fifty patients had a positive Ht balance with RCE (>2% mean increase from pre-procedure level), while 43 patients maintained a neutral balance. The first group required fewer red blood cell units/year (65 vs. 80, p < 0.001), but a significant proportion were iron overloaded based on R2* with liver MRI (32% vs. none performed) and prescription of iron chelation (52% vs. 0%, p < 0.001, after a median of 19 months). The second group was more likely to receive iron supplementation (6% vs. 56%, p < 0.001). Chronic automated RCE with a post-procedure Ht targeted at 34% is not iron-neutral, and personalized Ht goals may be more appropriate in certain settings. This higher target should be compared with a lower Ht strategy in individuals with similar baseline red cell volumes to assess iron homeostasis and blood product requirements.

摘要

在镰状细胞病的慢性管理中,红细胞置换(RCE)的最佳目标尚未明确界定。我们分析了101例接受慢性RCE治疗患者的输血需求和铁相关结局,这些患者术后血细胞比容(Ht)目标设定为34%,高于通常使用的水平。大多数患者为HbSS/HbSβ0基因型(n = 72),因神经系统并发症入组(n = 53)。50例患者RCE后Ht呈正向平衡(较术前水平平均增加>2%),而43例患者维持中性平衡。第一组每年所需红细胞单位较少(65 vs. 80,p < 0.001),但基于肝脏MRI的R2*和铁螯合剂处方,有相当比例的患者存在铁过载(32% vs. 未进行检测)(中位时间19个月后,52% vs. 0%,p < 0.001)。第二组更有可能接受铁补充(6% vs. 56%,p < 0.001)。术后Ht目标设定为34%的慢性自动RCE并非铁中性,在某些情况下,个性化的Ht目标可能更合适。应将这一较高目标与具有相似基线红细胞体积的个体采用较低Ht策略进行比较,以评估铁稳态和血液制品需求。

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