Shah Nirmish, Welsby Ian J, Fielder Martha A, Jacobsen Wayne K, Nielsen Vance G
Division of Hematology/Oncology, Department of Pediatrics, Duke University, Durham, NC, USA.
J Thromb Thrombolysis. 2015 Aug;40(2):182-5. doi: 10.1007/s11239-015-1230-6.
Sickle cell disease (SCD) is associated with a significant hypercoagulable state and several hemostatic anomalies have been identified in this disease state. Of interest, SCD patients can become iron overloaded after transfusion, and iron can enhance fibrinogen as a substrate for thrombin, resulting in thrombi that commence coagulation quickly and form rapidly. We hypothesized that SCD patients would display hypercoagulable plasma coagulation kinetics and an iron enhancement of coagulation. After obtaining IRB approval, we assessed coagulation kinetics and iron enhancement with viscoelastic methods in archived, citrated plasma obtained from ambulatory or hospitalized SCD patients (n = 20). All SCD patients had plasmatic hypercoagulability, and 65 % were positive for iron enhancement of coagulation. In conclusion, continuing investigation correlating such viscoelastic data with clinical symptoms may provide insight into the role played by iron in the setting of SCD, including complications such as vaso-occlusive crisis.
镰状细胞病（SCD）与显著的高凝状态相关，并且在这种疾病状态下已发现多种止血异常。有趣的是，SCD患者输血后可能会出现铁过载，铁可增强纤维蛋白原作为凝血酶的底物，导致血栓迅速启动凝血并快速形成。我们推测SCD患者会表现出高凝的血浆凝血动力学以及铁对凝血的增强作用。获得机构审查委员会（IRB）批准后，我们使用粘弹性方法评估了从门诊或住院的SCD患者（n = 20）获得的存档枸橼酸盐血浆中的凝血动力学和铁增强作用。所有SCD患者均具有血浆高凝性，65%的患者铁增强凝血试验呈阳性。总之，持续开展将此类粘弹性数据与临床症状相关联的研究，可能有助于深入了解铁在SCD发病过程中所起的作用，包括血管闭塞性危机等并发症。
