Atichartakarn Vichai, Chuncharunee Suporn, Archararit Napaporn, Udomsubpayakul Umaporn, Aryurachai Katcharin
Division of Hematology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Acta Haematol. 2014;132(1):100-7. doi: 10.1159/000355719. Epub 2014 Feb 11.
The relationship between asplenia and thrombophilia in β-thalassemia disease patients is not yet completely understood. One hundred and ten adult hemoglobin (Hb) E/β-thalassemia (E/β-Thal) disease outpatients, dichotomized according to the presence or absence of the spleen, were prospectively studied for evidence of intravascular hemolysis (IVH) and vascular endothelial cell (EC) activation. Biomarkers of IVH (serum cell-free Hb), EC [soluble E-selectin (sE-selectin) and soluble vascular cell adhesion molecule 1 (sVCAM-1)], platelet and EC [soluble P-selectin (sP-selectin)], inflammation [high-sensitivity C-reactive protein (hs-CRP)], and coagulation [thrombin-antithrombin complexes (TAT)] activation, as well as other selected blood tests were determined. The 61 splenectomized patients had a more severe hemolytic disease and higher levels of cell-free Hb and ferritin (p = 0.003), sE-selectin, sP-selectin, hs-CRP, and TAT (p < 0.05). However, serum levels of sVCAM-1 were not different between the two groups. The findings suggested IVH and EC activation. Together with chronic iron overload and chronic low-grade inflammation activation, the findings extend our understanding of the mechanism of thrombophilia in splenectomized E/β-Thal disease patients.
β地中海贫血病患者无脾与血栓形成倾向之间的关系尚未完全明确。110例成年血红蛋白（Hb）E/β地中海贫血（E/β-Thal）病门诊患者，根据有无脾脏分为两组，前瞻性研究血管内溶血（IVH）和血管内皮细胞（EC）激活的证据。检测IVH的生物标志物（血清游离Hb）、EC [可溶性E选择素（sE-selectin）和可溶性血管细胞黏附分子1（sVCAM-1）]、血小板和EC [可溶性P选择素（sP-selectin）]、炎症[高敏C反应蛋白（hs-CRP）]和凝血[凝血酶-抗凝血酶复合物（TAT）]激活情况，以及其他选定的血液检查项目。61例脾切除患者的溶血性疾病更严重，游离Hb和铁蛋白水平更高（p = 0.003），sE-selectin、sP-selectin、hs-CRP和TAT水平更高（p < 0.05）。然而，两组间sVCAM-1的血清水平无差异。这些发现提示存在IVH和EC激活。连同慢性铁过载和慢性低度炎症激活，这些发现扩展了我们对脾切除E/β-Thal病患者血栓形成倾向机制的理解。
