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Manual erythroexchange in sickle cell disease: multicenter validation of a protocol predictive of volume to exchange and hemoglobin values.

镰状细胞病的手动红细胞交换: 预测交换量和血红蛋白值的方案的多中心验证。

  • 影响因子:1.94
  • DOI:10.1007/s00277-020-04188-y
  • 作者列表:"Gianesin B","Pinto VM","Casale M","Corti P","Fidone C","Quintino S","Voi V","Forni GL
  • 发表时间:2020-09-01
Abstract

:Manual erythroexchange (MEEX) was proven to be effective and safe in the management of sickle cell disease (SCD). The goal is to quickly reduce the percentage of hemoglobin S (HbS%). A national survey of the Italian Society for Thalassemia and Hemoglobinopathies (SITE) observed a great variability among MEEX protocols none of which were found to be predictive of the values of HbS% and hemoglobin (Hb) after the exchange. Two equations to estimate the HbS% and Hb values to be obtained after MEEX were developed based on the results of the MEEX procedures in place in the centers participating in the present study. A standard protocol was subsequently defined to evaluate the volumes to exchange to obtain the target values of HbS% and Hb. The protocol was tested in 261 MEEX performed in SCD patients followed in the 5 participating centers that belong to the Italian Hemoglobinopathy Comprehensive Care Network, with the support of the SITE. The results showed a correlation between the estimated and measured values of HbS% and Hb (Rp 0.95 and 0.65 respectively, p < 0.001). A negligible bias was found for the prediction of HbS% and a bias of 1 g/dl for Hb. From consecutive MEEX, a rate of increase of HbS% between two exchanges of around 0.4% per day (p < 0.001) was measured. This protocol was shown to be effective and safe, as all patients reached the target value of HbS%. All the MEEX procedures were carried out with single venous access. No adverse events or reactions such as hypotension or electrolyte imbalance were reported nor were any complaints concerning the procedures received from patients.

摘要

: 手动红细胞交换 (MEEX) 被证明在镰状细胞病 (SCD) 的管理中是有效和安全的。目标是快速降低血红蛋白S的百分比 (HbS %)。意大利地中海贫血和血红蛋白病协会 (SITE) 的一项全国调查观察到MEEX方案之间存在很大差异,没有发现这些方案可以预测交换后HbS % 和血红蛋白 (Hb) 的值。基于参与本研究的中心的MEEX程序的结果,开发了两个方程来估计在MEEX之后获得的HbS % 和Hb值。随后定义标准方案以评估交换体积以获得HbS % 和Hb的目标值。在该网站的支持下,在意大利血红蛋白病综合护理网络的5个参与中心的SCD患者中进行了261例MEEX的试验.结果显示,HbS % 和Hb的估计值和测量值之间存在相关性 (Rp分别为0.95和0.65,p <0.001)。对于HbS % 的预测发现可忽略的偏差,对于Hb发现1g/dl的偏差。从连续的MEEX开始,测量两次交换之间HbS % 的增加率为每天约0.4% (p <0.001)。该方案被证明是有效和安全的,因为所有患者都达到HbS % 的目标值。所有MEEX程序均采用单次静脉通路进行。未报告不良事件或反应,如低血压或电解质失衡,也未收到患者对手术的任何投诉。

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翻译标题与摘要 下载文献
血红蛋白病方向

由于血红蛋白分子结构异常(异常血红蛋白病),或珠蛋白肽链合成速率异常(珠蛋白生成障碍性贫血,又称海洋性贫血)所引起的一组遗传性血液病。

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