Kanavaki Ino, Makrythanasis Periklis, Lazaropoulou Christina, Tsironi Maria, Kattamis Antonis, Rombos Ioannis, Papassotiriou Ioannis
Department of Clinical Biochemistry, Aghia Sophia Children's Hospital, 115 27 Athens, Greece.
Blood Cells Mol Dis. 2009 Nov-Dec;43(3):230-4. doi: 10.1016/j.bcmd.2009.06.002. Epub 2009 Aug 4.
The term thalassemia intermedia, indicates a clinical condition of intermediate severity between thalassaemia minor, the asymptomatic carrier, and thalassaemia major, the transfusion-dependent, severe form. Thromboembolic events frequently complicate the outcome of thalassemia intermedia patients, reflecting a hypercoagulable state to which endothelial activation is believed to play an important role. The aim of this study was to evaluate the levels of soluble endothelial adhesion molecules that reflect endothelial activation and dysfunction and levels of chronic inflammation markers in the serum of beta-thalassemia intermedia patients. Thirty-five Greek patients with beta-thalassemia intermedia that have received different types of treatment (Hydroxyurea, splenectomy, untreated), aged 8-63 years, were included in the study. Twenty apparently healthy individuals matched for age and sex, formed the control group. Measurements of sVCAM-1, sICAM-1, sTM, P-selectin, E-selectin and CRP levels were performed using immunoassays. We found that all endothelial adhesion molecules and CRP were significantly increased in patients (p<0.001) and not influenced by treatment. A negative correlation was observed between levels of sICAM-1 and sTM and this finding agrees with the results of studies, which propose this correlation as a predictive marker of increased risk for vascular damage. No correlation was observed between endothelial adhesion molecules and inflammation markers. These findings support the hypothesis that a serious degree of endothelial activation and damage along with a state of chronic inflammation underlie the pathophysiology of beta-thalassemia intermedia. Furthermore, these findings are of particular importance in patients who can otherwise be characterized by a subtle clinical phenotype and may have an important role in their clinical care.
中间型地中海贫血这一术语，指的是一种临床严重程度介于轻型地中海贫血（无症状携带者）和重型地中海贫血（依赖输血的严重形式）之间的病症。血栓栓塞事件常使中间型地中海贫血患者的病情复杂化，反映出一种高凝状态，而内皮激活被认为在其中起重要作用。本研究的目的是评估反映内皮激活和功能障碍的可溶性内皮黏附分子水平以及中间型β地中海贫血患者血清中的慢性炎症标志物水平。35名年龄在8至63岁之间、接受过不同类型治疗（羟基脲、脾切除术、未治疗）的希腊中间型β地中海贫血患者被纳入研究。20名年龄和性别匹配的明显健康个体组成对照组。使用免疫测定法测量可溶性血管细胞黏附分子-1（sVCAM-1）、可溶性细胞间黏附分子-1（sICAM-1）、可溶性血栓调节蛋白（sTM）、P-选择素、E-选择素和C反应蛋白（CRP）水平。我们发现，患者体内所有内皮黏附分子和CRP均显著升高（p<0.001），且不受治疗影响。观察到sICAM-1和sTM水平之间呈负相关，这一发现与一些研究结果一致，这些研究提出这种相关性可作为血管损伤风险增加的预测标志物。未观察到内皮黏附分子与炎症标志物之间存在相关性。这些发现支持了这样一种假说，即严重程度的内皮激活和损伤以及慢性炎症状态是中间型β地中海贫血病理生理学的基础。此外，这些发现在那些原本可能具有细微临床表型的患者中尤为重要，并且可能在他们的临床护理中发挥重要作用。
