Hemoglobin disorders and endothelial cell interactions

被引:45
|
作者
Conran, Nicola [1 ]
Costa, Fernando F. [1 ]
机构
[1] Univ Estadual Campinas, Sch Med Sci, Hematol & Hemotherapy Ctr, BR-13083970 Campinas, SP, Brazil
基金
巴西圣保罗研究基金会;
关键词
Endothelial cells; Inflammation; Sickle cell disease; Thalassemia; Vaso-occlusion; Pathophysiology; RED-BLOOD-CELLS; PULMONARY ARTERIAL-HYPERTENSION; TRANSGENIC SICKLE MICE; TUMOR-NECROSIS-FACTOR; INHALED NITRIC-OXIDE; ACUTE-CHEST-SYNDROME; UP-REGULATES ICAM-1; VASCULAR ENDOTHELIUM; BETA-THALASSEMIA; OXIDATIVE STRESS;
D O I
10.1016/j.clinbiochem.2009.06.024
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
Endothelial damage and inflammation make a significant contribution to the pathophysiology of sickle cell disease (SCD) and the beta-thalassemia syndromes. Endothelial dysfunction and ensuing vasculopathy are implicated in pulmonary hypertension in the hemoglobinopathies and endothelial activation and endothelial-blood cell adhesion, accompanied by inflammatory processes and oxidative stress, are imperative to the vaso-occlusive process in SCD. Herein, we discuss the role that the endothelium plays in all of these processes and the effect that genetic modifiers and hydroxyurea therapy may have upon endothelial interactions. Therapies targeting the endothelium and endothelial interactions may represent a promising approach for treating these diseases. (C) 2009 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.
引用
收藏
页码:1824 / 1838
页数:15
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