Division of Paediatric Cardiology, Department of Paediatrics and Adolescent Medicine, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China.
Ann Hematol. 2012 Mar;91(3):345-52. doi: 10.1007/s00277-011-1302-4. Epub 2011 Aug 2.
Arterial dysfunction has been documented in patients with beta-thalassaemia major. This study aimed to determine the quantity and proliferative capacity of circulating CD133(+)VEGFR2(+) and CD34(+)VEGFR2(+) cells in patients with beta-thalassaemia major and those after haematopoietic stem cell transplantation (HSCT), and their relationships with arterial function. Brachial arterial flow-mediated dilation (FMD), carotid arterial stiffness, the quantity of these circulating cells and their number of colony-forming units (CFUs) were determined in 17 transfusion-dependent thalassaemia patients, 14 patients after HSCT and 11 controls. Compared with controls, both patient groups had significantly lower FMD and greater arterial stiffness. Despite having increased CD133(+)VEGFR2(+) and CD34(+)VEGFR2(+) cells, transfusion-dependent patients had significantly reduced CFUs compared with controls (p = 0.002). There was a trend of increasing CFUs across the three groups with decreasing iron load (p = 0.011). The CFUs correlated with brachial FMD (p = 0.029) and arterial stiffness (p = 0.02), but not with serum ferritin level. Multiple linear regression showed that CFU was a significant determinant of FMD (p = 0.043) and arterial stiffness (p = 0.02) after adjustment of age, sex, body mass index, blood pressure and serum ferritin level. In conclusion, arterial dysfunction found in patients with beta-thalassaemia major before and after HSCT may be related to impaired proliferation of CD133(+)VEGFR2(+) and CD34(+)VEGFR2(+) cells.
动脉功能障碍已在β地中海贫血患者中得到证实。本研究旨在确定β地中海贫血患者和造血干细胞移植（HSCT）后的患者循环 CD133(+)VEGFR2(+)和 CD34(+)VEGFR2(+)细胞的数量和增殖能力，并研究其与动脉功能的关系。本研究共纳入 17 名依赖输血的地中海贫血患者、14 名 HSCT 后患者和 11 名对照者，检测其肱动脉血流介导的舒张功能（FMD）、颈动脉硬度、这些循环细胞的数量及其集落形成单位（CFU）。与对照组相比，两组患者的 FMD 均显著降低，动脉僵硬程度均显著增加。尽管依赖输血的患者 CD133(+)VEGFR2(+)和 CD34(+)VEGFR2(+)细胞数量增加，但 CFU 显著低于对照组（p = 0.002）。随着铁负荷的降低，三组患者的 CFU 呈增加趋势（p = 0.011）。CFU 与肱动脉 FMD（p = 0.029）和动脉僵硬（p = 0.02）呈正相关，但与血清铁蛋白水平无关。多元线性回归显示，校正年龄、性别、体重指数、血压和血清铁蛋白水平后，CFU 是 FMD（p = 0.043）和动脉僵硬（p = 0.02）的显著决定因素。综上，β地中海贫血患者 HSCT 前后动脉功能障碍可能与 CD133(+)VEGFR2(+)和 CD34(+)VEGFR2(+)细胞增殖受损有关。
