Novel Red Blood Cell Exchange Parameters for Treatment of Transfusion-Dependent Thalassemia Based on Experience of Five Patients.

IF 1.3 Q4 HEMATOLOGY
Journal of hematology Pub Date : 2025-07-08 eCollection Date: 2025-08-01 DOI:10.14740/jh2086
Kristina Sevcik, Claire Jackson, Shelly M Williams, Scott A Koepsell, Aleh Bobr
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引用次数: 0

Abstract

Background: Thalassemias are inherited red blood cell disorders characterized by defective globin production, resulting in microcytic hypochromic anemia. Severe variants lead to transfusion dependence and consequent iron overload, often despite chelation therapy. The role of automated red blood cell exchange (RBCX) for transfusion-dependent thalassemia (TDT) is unclear and previously there was no specific apheresis parameters specific for thalassemia defined. We present our experience with patients with TDT treated with RBCX using higher hematocrit parameters specifically tailored for this condition.

Methods: Five patients with TDT underwent chronic RBCX in place of simple transfusion with the primary goal of stabilizing iron overload. Novel parameters were established to satisfy the Thalassemia International Federation goal pre-transfusion hemoglobin of 9.5 g/dL, while targeting a post-transfusion hematocrit of 37-38%.

Results: RBCX was well tolerated with infrequent occurrences of vasovagal reactions, asymptomatic hypotension, citrate side effects, and vascular access issues. The transfusion interval increased from an average of 3 weeks with simple transfusions to 5 weeks with RBCX. Despite an increase in average blood utilization, serum ferritin levels remained stable.

Conclusion: RBCX with higher hematocrit parameters can be performed safely and efficiently in TDT patients. To our knowledge, this is the first report of TDT-specific RBCX parameters. Though blood utilization is higher with RBCX, it offers longer intervals between transfusions and has no increase in iron overload, improving quality of life for patients.

Abstract Image

基于5例输血依赖性地中海贫血患者经验的新红细胞交换参数。
背景:地中海贫血是一种遗传性红细胞疾病,其特征是珠蛋白产生缺陷,导致小细胞性低色性贫血。严重的变异导致输血依赖和随之而来的铁超载,通常尽管螯合治疗。自动红细胞交换(RBCX)在输血依赖性地中海贫血(TDT)中的作用尚不清楚,以前没有针对地中海贫血定义的特异性采血参数。我们介绍了我们使用RBCX治疗TDT患者的经验,RBCX使用专门为这种情况量身定制的更高的红细胞压积参数。方法:5例TDT患者接受慢性RBCX代替单纯输血,主要目的是稳定铁超载。建立了新的参数,以满足地中海贫血国际联合会的输血前血红蛋白9.5 g/dL的目标,而输血后红细胞压积为37-38%。结果:RBCX耐受性良好,很少发生血管迷走反应、无症状性低血压、柠檬酸盐副作用和血管通路问题。输血间隔从单纯输血的平均3周增加到RBCX的5周。尽管平均血液利用率增加,血清铁蛋白水平保持稳定。结论:较高红细胞压积参数的RBCX可安全有效地用于TDT患者。据我们所知,这是关于tdt特定RBCX参数的第一份报告。虽然RBCX的血液利用率较高,但输血间隔时间较长,铁负荷没有增加,提高了患者的生活质量。
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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
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