Colella Marina P, de Paula Erich V, Machado-Neto João A, Conran Nicola, Annichino-Bizzacchi Joyce M, Costa Fernando F, Olalla Saad Sara T, Traina Fabiola
Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas.
Hematology and Hemotherapy Center - University of Campinas/Hemocentro UNICAMP, Instituto Nacional de Ciência e Tecnologia do Sangue, Campinas Currently at the Department of Internal Medicine, University of São Paulo at Ribeirão Preto Medical School, São Paulo, Brazil
Haematologica. 2015 Apr;100(4):466-71. doi: 10.3324/haematol.2014.114587. Epub 2015 Jan 16.
Hemoglobin SC disease is a very prevalent hemoglobinopathy; however, very little is known about this condition specifically. There appears to be an increased risk of thromboembolic events in hemoglobin SC disease, but studies evaluating the hemostatic alterations are lacking. We describe the findings of a cross-sectional observational study evaluating coagulation activation markers in adult patients with hemoglobin SC, comparing them with those in sickle cell anemia patients and healthy controls. A total of 56 hemoglobin SC and 39 sickle cell anemia patients were included in the study, all in steady state, and 27 healthy controls. None of the patients was taking hydroxyurea. Hemoglobin SC patients had a significantly up-regulated relative expression of tissue factor, as well as elevations in thrombin-antithrombin complex and D-dimer, in comparison to controls (P<0.01). Hemoglobin SC patients had lower tissue factor expression, and thrombin-antithrombin complex and D-dimer levels when compared to sickle cell anemia patients (P<0.05). Markers of endothelial activation (soluble thrombomodulin and soluble vascular cell adhesion molecule-1) and inflammation (tumor necrosis factor-alpha) were both significantly elevated in hemoglobin SC patients when compared to controls, being as high as the levels seen in patients with sickle cell anemia. Overall, in hemoglobin SC patients, higher hemolytic activity and inflammation were associated with a more intense activation of coagulation, and hemostatic activation was associated with two very prevalent chronic complications seen in hemoglobin SC disease: retinopathy and osteonecrosis. In summary, our results demonstrate that hemoglobin SC patients have a hypercoagulable state, although this manifestation was not as intense as that seen in sickle cell anemia.
血红蛋白SC病是一种非常常见的血红蛋白病；然而，人们对这种疾病的具体情况知之甚少。血红蛋白SC病患者发生血栓栓塞事件的风险似乎有所增加，但缺乏评估止血改变的研究。我们描述了一项横断面观察性研究的结果，该研究评估了成年血红蛋白SC病患者的凝血激活标志物，并将其与镰状细胞贫血患者和健康对照者的标志物进行比较。该研究共纳入了56例血红蛋白SC病患者和39例镰状细胞贫血患者，所有患者均处于稳定状态，另有27名健康对照者。所有患者均未服用羟基脲。与对照组相比，血红蛋白SC病患者组织因子的相对表达显著上调，凝血酶-抗凝血酶复合物和D-二聚体水平也升高（P<0.01）。与镰状细胞贫血患者相比，血红蛋白SC病患者的组织因子表达、凝血酶-抗凝血酶复合物和D-二聚体水平较低（P<0.05）。与对照组相比，血红蛋白SC病患者的内皮激活标志物（可溶性血栓调节蛋白和可溶性血管细胞黏附分子-1）和炎症标志物（肿瘤坏死因子-α）均显著升高，与镰状细胞贫血患者的水平相当。总体而言，在血红蛋白SC病患者中，较高的溶血活性和炎症与更强烈的凝血激活相关，而止血激活与血红蛋白SC病中两种非常常见的慢性并发症有关：视网膜病变和骨坏死。总之，我们的结果表明，血红蛋白SC病患者存在高凝状态，尽管这种表现不如镰状细胞贫血患者明显。
