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细胞外囊泡在β-地中海贫血临床并发症发生中的作用。

The role of extracellular vesicles on the occurrence of clinical complications in β-thalassemia.

机构信息

Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Iranian Blood Transfusion Organization (IBTO), Tehran, Iran; Ramsar Campus, Mazandaran University of Medical Sciences, Ramsar, Iran.

Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Iranian Blood Transfusion Organization (IBTO), Tehran, Iran.

出版信息

Exp Hematol. 2023 Nov;127:28-39. doi: 10.1016/j.exphem.2023.08.009. Epub 2023 Aug 29.

Abstract

Thalassemia is the most common monogenic disorder of red blood cells (RBCs) caused by defects in the synthesis of globin chains. Thalassemia phenotypes have a wide spectrum of clinical manifestations and vary from severe anemia requiring regular blood transfusions to clinically asymptomatic states. Ineffective erythropoiesis and toxicity caused by iron overload are major factors responsible for various complications in thalassemia patients, especially patients with β-thalassemia major (β-TM). Common complications in patients with thalassemia include iron overload, thrombosis, cardiac morbidity, vascular dysfunction, inflammation, and organ dysfunction. Extracellular vesicles (EVs) are small membrane vesicles released from various cells' plasma membranes due to activation and apoptosis. Based on studies, EVs play a role in various processes, including clot formation, vascular damage, and proinflammatory processes. In recent years, they have also been studied as biomarkers in the diagnosis and prognosis of diseases. Considering the high concentration of EVs in thalassemia and their role in cellular processes, this study reviews the role of EVs in the common complications of patients with β-thalassemia for the first time.

摘要

地中海贫血是最常见的单基因红细胞(RBC)疾病,由珠蛋白链合成缺陷引起。地中海贫血表型具有广泛的临床表现,从需要定期输血的严重贫血到临床无症状状态不等。无效的红细胞生成和铁过载引起的毒性是导致地中海贫血患者发生各种并发症的主要因素,尤其是重型β地中海贫血(β-TM)患者。地中海贫血患者的常见并发症包括铁过载、血栓形成、心脏发病、血管功能障碍、炎症和器官功能障碍。细胞外囊泡(EVs)是由各种细胞的质膜由于激活和凋亡而释放的小膜囊泡。基于研究,EVs 在包括血栓形成、血管损伤和促炎过程在内的各种过程中发挥作用。近年来,它们也被作为疾病诊断和预后的生物标志物进行研究。鉴于 EVs 在地中海贫血中的高浓度及其在细胞过程中的作用,本研究首次综述了 EVs 在β地中海贫血患者常见并发症中的作用。

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