Hemoglobinopathies and Congenital Anemia Center, Ospedale Galliera, Genoa, Italy.
Department of Woman Child and General Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
Ann Hematol. 2020 Sep;99(9):2047-2055. doi: 10.1007/s00277-020-04188-y. Epub 2020 Jul 21.
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 (R 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.
手工红细胞交换（MEEX）已被证明可有效且安全地治疗镰状细胞病（SCD）。其目的是快速降低血红蛋白 S（HbS%）的百分比。意大利地中海贫血和血红蛋白病学会（SITE）的一项全国性调查观察到，MEEX 方案之间存在很大差异，没有一个方案可以预测交换后 HbS%和血红蛋白（Hb）的值。根据参与本研究的中心进行的 MEEX 程序的结果，制定了两个方程来估计 MEEX 后要获得的 HbS%和 Hb 值。随后制定了一个标准方案来评估要交换的体积，以获得 HbS%和 Hb 的目标值。该方案在意大利血红蛋白病综合护理网络所属的 5 个参与中心的 SCD 患者中进行了 261 次 MEEX 测试，得到了 SITE 的支持。结果表明，HbS%和 Hb 的估计值与实测值之间存在相关性（分别为 R 0.95 和 0.65，p<0.001）。对于 HbS%的预测，发现了可以忽略不计的偏差，而对于 Hb，预测值存在 1 克/分升的偏差。从连续的 MEEX 中，两次交换之间 HbS%的增长率约为每天 0.4%（p<0.001）。该方案被证明是有效且安全的，因为所有患者均达到 HbS%的目标值。所有 MEEX 程序均通过单次静脉通路进行。没有发生低血压或电解质失衡等不良反应或事件，也没有收到患者对程序的任何投诉。
