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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.

DOI:10.1111/bjh.19674
PMID:39081092
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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