Nsiri B, Gritli N, Bayoudh F, Messaoud T, Fattoum S, Machghoul S
Laboratoire d'Hématologie, Hôpital Militaire de Tunis, Montfleury, Tunisie.
Hematol Cell Ther. 1996 Jul;38(3):279-84. doi: 10.1007/s00282-996-0279-2.
Abnormalities of coagulation and fibrinolysis were studied in a group of 28 children and young adults with homozygous sickle cell disease (SCD), either in the steady state (n = 12) or during painful crisis (n = 16). Coagulation was explored by standard clotting tests and by measurement of prothrombin complex factors, factor VIII (VIII:C) and antithrombin III (ATIII), protein C (PC) and protein S (PS) activities, while fibrinolytic potential was evaluated using D-dimer, tissue plasminogen activator (t-PA) and plasminogen activator inhibitor (PAI-1) assays. In SCD patients, thrombin time (TT) was constantly shortened, both in the steady state (ratio to control 0.83 +/- 0.08, p < 0.0001) and in crisis (0.76 +/- 0.06, p < 0.0001). Mean levels of prothrombin complex were similar in asymptomatic patients to those in controls, but were significantly decreased during sickle cell crisis (p < 0.05 for factor V and p < 0.0001 for factors II, VII and X). Factor VIII:C was significantly increased, both in the steady state (207 +/- 35%, p < 0.0001) and during crisis (208 +/- 34%, p < 0.0001). PS activity was reduced int he steady state (81 +/- 12%, p < 0.01) and further diminished in crisis (68.5 +/- 27.5%, p < 0.001), while D-dimers were significantly elevated during sickle cell crisis (1028 +/- 675 ng/ml, p < 0.001). In all SCD patients, baseline levels of t-PA antigen were comparable to those in controls, whereas concentrations of PAI-1 antigen were significantly increased, either in the steady state (89.7 +/- 26.3 ng/ml, p < 0.0001) or in crisis (75.0 +/- 24.8 ng/ml, p < 0.0001). These results provide evidence for the presence of circulating activated clotting factors in SCD and for an imbalance of the profibrinolytic and antifibrinolytic systems most likely due to increased PAI-1 levels.
对一组28名患有纯合子镰状细胞病（SCD）的儿童和青年进行了凝血和纤维蛋白溶解异常的研究，这些患者处于稳定状态（n = 12）或疼痛危象期间（n = 16）。通过标准凝血试验以及测量凝血酶原复合因子、因子VIII（VIII:C）和抗凝血酶III（ATIII）、蛋白C（PC）和蛋白S（PS）活性来探究凝血情况，同时使用D - 二聚体、组织纤溶酶原激活物（t - PA）和纤溶酶原激活物抑制剂（PAI - 1）检测来评估纤维蛋白溶解潜能。在SCD患者中，凝血酶时间（TT）持续缩短，在稳定状态下（与对照组的比值为0.83±0.08，p < 0.0001）以及危象期间（0.76±0.06，p < 0.0001）均如此。无症状患者的凝血酶原复合因子平均水平与对照组相似，但在镰状细胞危象期间显著降低（因子V p < 0.05，因子II、VII和X p < 0.0001）。因子VIII:C显著升高，在稳定状态下（207±35%，p < 0.0001）以及危象期间（208±34%，p < 0.0001）均是如此。PS活性在稳定状态下降低（81±12%，p < 0.01），在危象期间进一步降低（68.5±27.5%，p < 0.001），而D - 二聚体在镰状细胞危象期间显著升高（1028±675 ng/ml，p < 0.001）。在所有SCD患者中，t - PA抗原的基线水平与对照组相当，而PAI - 1抗原的浓度显著升高，无论是在稳定状态下（89.7±26.3 ng/ml，p < 0.0001）还是在危象期间（75.0±24.8 ng/ml，p < 0.0001）。这些结果为SCD中存在循环活化凝血因子以及纤维蛋白溶解和抗纤维蛋白溶解系统失衡提供了证据，这种失衡很可能是由于PAI - 1水平升高所致。
