Sanquin Research and Landsteiner Laboratory, Immunopathology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands.
Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Pediatric Hematology, Amsterdam, the Netherlands.
Transfusion. 2022 Oct;62(10):1984-1996. doi: 10.1111/trf.17033. Epub 2022 Aug 2.
Red blood cell (RBC) transfusions are an important treatment modality for patients with sickle cell disease (SCD) and β-thalassemia. A subgroup of these patients relies on a chronic RBC transfusion regimen. Little is known about RBC survival (RCS) of the transfused allogeneic RBCs. In this study, we aimed to study the RCS kinetics of transfused RBCs in SCD and β-thalassemia and to investigate factors that determine RCS.
We performed a prospective cohort study on fourteen adults with SCD and β-thalassemia disease receiving a chronic transfusion regimen. RCS and the influence of donor and patient characteristics on RCS were assessed by simultaneous transfusion of two allogeneic RBCs using RBC biotinylation. Phenotyping of well-known RBC markers over time was performed using flow cytometry.
RCS of the two transfused RBC units was similar in most patients. Although intra-individual variation was small, inter-individual variation in RCS kinetics was observed. Most patients demonstrated a non-linear trend in RCS that was different from the observed linear RCS kinetics in healthy volunteers. After an initial slight increase in the proportion of biotinylated RBCs during the first 24 h, a rapid decrease within the first 10-12 days was followed by a slower clearance rate.
These are the first data to demonstrate that patient-related factors largely determine post-transfusion RCS behavior of donor RBC in SCD and β-thalassemia, while donor factors exert a negligible effect. Further assessment and modeling of RCS kinetics and its determinants in SCD and β-thalassemia patients may ultimately improve transfusion therapy.
红细胞（RBC）输血是治疗镰状细胞病（SCD）和β-地中海贫血患者的重要治疗方法。这些患者中有一部分依赖于慢性 RBC 输血方案。对于输注的同种异体 RBC 的 RBC 存活（RCS）知之甚少。在这项研究中，我们旨在研究 SCD 和β-地中海贫血患者输注 RBC 的 RCS 动力学，并研究决定 RCS 的因素。
我们对 14 名接受慢性输血方案的 SCD 和β-地中海贫血成年患者进行了前瞻性队列研究。通过使用 RBC 生物素化同时输注两种同种异体 RBC 来评估 RCS 及其对 RCS 的影响。使用流式细胞术随时间推移对已知 RBC 标志物的表型进行了评估。
在大多数患者中，两种输注的 RBC 单位的 RCS 相似。尽管个体内差异较小，但观察到 RCS 动力学的个体间差异。大多数患者表现出 RCS 的非线性趋势，与健康志愿者观察到的线性 RCS 动力学不同。在最初的 24 小时内，生物素化 RBC 的比例略有增加后，在最初的 10-12 天内迅速下降，然后清除率较慢。
这些是首次证明患者相关因素在很大程度上决定 SCD 和β-地中海贫血患者输注 RBC 的 RCS 行为，而供体因素的影响可以忽略不计。进一步评估和建模 SCD 和β-地中海贫血患者的 RCS 动力学及其决定因素最终可能会改善输血治疗。
