Rość D, Kremplewska-Nalezyta E, Gadomska G, Zastawna E, Michalski A, Drewniak W
Department of Pathophysiology, Medical University, ul. M. Skłodowskiej-Curie 9, 85-094 Bydgoszcz, Poland.
Med Sci Monit. 2000 Jul-Aug;6(4):684-91.
Clinical observations and laboratory data indicate that patients with myeloproliferative syndrome (MPS) have hemostasis disorders that manifest with haemorrhagic diathesis or thrombotic disease. The role of fibrinolytic system is not clear in the pathomechanism of the disorders. In the study we aimed to evaluate fibrinolysis system in blood plasma of the patients with selected myeloproliferative syndromes on the basis of examinations of plasminogen activators (tissue--t-PA and urokinase-type--u-PA) and type 1 and type 2 plasminogen activator inhibitors (PAI-1 and PAI-2).
Forty-two patients (F/M 28/14) aged 36-78 years (average age--54 years) were enrolled into the study. Among the patients were 20 individuals with chronic myeloid leukaemia (CML), 17 with essential thrombocytemia (ET) and 5 with myelofibrosis (MF) treated in Provincial Haematological Outpatient Clinic in Bydgoszcz. Forty healthy volunteers (F/M 30/10) aged 24-65 (average 53) were included in control group. In citrate venous blood, the following parameters were determined: concentrations of antigen t-PA, u-PA, PAI-1, PAI-2, concentration of plasmin-alpha-2-antiplasmin complexes (PAP) determined with the use of ELISA technique, PAI-1 activity with amidolytic method, euglobulin lysis time (ELT) according to Kowarzyk-Buluk and fibrinogen/fibrin degradation products (FDP) concentration with the use of Merskey's method.
Performed evaluations show that besides normal ELT elevated concentrations of FDP and PAP complexes occur in the blood of patients with myeloproliferative syndromes (MPS.). In studied myeloproliferative syndromes, high concentration of PAI-1 Ag and very low activity of PAI-1 were observed. In the patients with MPS intensified plasminogenesis and increased fibrin degradation were demonstrated. The role of granulocyte elastase is considered in activation of fibrinolysis system.
临床观察和实验室数据表明，骨髓增殖综合征（MPS）患者存在止血障碍，表现为出血素质或血栓性疾病。纤溶系统在这些疾病的发病机制中的作用尚不清楚。在本研究中，我们旨在通过检测纤溶酶原激活剂（组织型 - t-PA和尿激酶型 - u-PA）以及1型和2型纤溶酶原激活剂抑制剂（PAI-1和PAI-2），评估特定骨髓增殖综合征患者血浆中的纤溶系统。
42例年龄在36 - 78岁（平均年龄54岁）的患者（女性28例/男性14例）纳入本研究。其中20例为慢性粒细胞白血病（CML）患者，17例为原发性血小板增多症（ET）患者，5例为骨髓纤维化（MF）患者，均在比得哥什省血液门诊接受治疗。40名年龄在24 - 65岁（平均53岁）的健康志愿者（女性30例/男性10例）作为对照组。在枸橼酸盐抗凝静脉血中，测定以下参数：抗原t-PA、u-PA、PAI-1、PAI-2的浓度，使用ELISA技术测定纤溶酶-α2-抗纤溶酶复合物（PAP）的浓度，用酰胺水解法测定PAI-1活性，根据Kowarzyk-Buluk法测定优球蛋白溶解时间（ELT），以及使用Merskey法测定纤维蛋白原/纤维蛋白降解产物（FDP）浓度。
所进行的评估表明，除了正常的ELT外，骨髓增殖综合征（MPS）患者血液中FDP和PAP复合物的浓度升高。在所研究的骨髓增殖综合征中，观察到PAI-1抗原浓度高而PAI-1活性非常低。在MPS患者中，证实了纤溶酶生成增强和纤维蛋白降解增加。粒细胞弹性蛋白酶在纤溶系统激活中的作用也得到了考虑。
