Toschi V, Fiorini G F, Motta A, Renoldi P, Paracchini M L, Gibelli A
Department of Hematology and Blood Transfusion, Ospedale S. Carlo Borromeo, Milan, Italy.
Acta Haematol. 1991;86(2):90-4. doi: 10.1159/000204810.
Essential mixed cryoglobulinemia (EMC) is a rheumatic disorder characterized by widespread vasculitis. To better define the nature of the vasculitic process and to possibly outline assessment methods reliable for using in a clinical context, we studied plasma levels of three endothelial related peptides: fibronectin (FN), von Willebrand factor (vWF) and tissue plasminogen activator (t-PA), and those of thrombin-antithrombin III complexes (TAT) as markers of activation of the coagulation in 21 patients and in 16 controls. In EMC we found a picture consisting of reduced FN and increased vWF, t-PA, and TAT levels, suggesting a condition of endothelial cell damage with thrombin formation in vivo. Since we previously demonstrated the presence of chronic disseminated intravascular coagulation in these patients, we may assume that endothelial cells stressed by cryoprecipitation or stimulated by soluble mediators may be actively involved in the vasculitic process and possibly express procoagulant properties. This is a good example of the complex interplay existing between autoimmunity and coagulation mechanisms. We also suggest that FN, vWF, t-PA and TAT should be considered as additional clinical parameters when evaluating patients with EMC.
原发性混合性冷球蛋白血症（EMC）是一种以广泛血管炎为特征的风湿性疾病。为了更好地界定血管炎过程的本质，并可能勾勒出在临床环境中可靠使用的评估方法，我们研究了21例患者和16例对照者血浆中三种内皮相关肽：纤连蛋白（FN）、血管性血友病因子（vWF）和组织型纤溶酶原激活剂（t-PA）的水平，以及凝血酶 - 抗凝血酶III复合物（TAT）的水平，将其作为凝血激活的标志物。在EMC患者中，我们发现纤连蛋白水平降低，而vWF、t-PA和TAT水平升高，这表明体内存在内皮细胞损伤并伴有凝血酶形成的情况。由于我们之前已证实在这些患者中存在慢性弥散性血管内凝血，我们可以推测，受冷沉淀应激或可溶性介质刺激的内皮细胞可能积极参与血管炎过程，并可能表现出促凝血特性。这是自身免疫与凝血机制之间复杂相互作用的一个很好的例子。我们还建议，在评估EMC患者时，应将FN、vWF、t-PA和TAT视为额外的临床参数。
