Oudijk E J, Nieuwenhuis H K, Bos R, Fijnheer R
Department of Haematology, University Medical Centre, Utrecht, The Netherlands.
Thromb Haemost. 2000 Jun;83(6):906-8.
The bleeding syndrome of acute promyelocytic leukemia (APL) is complex and consists of disseminated intravascular coagulation (DIC) and hyperfibrinolysis. Elastase, derived from malignant promyelocytes, is believed to mediate the fibrinogeno- and fibrinolysis by aspecific proteolysis. In this study we measured the role of elastase in fifteen patients with APL by using an assay for elastase degraded fibrin(ogen) and the results were compared with those obtained in patients with sepsis induced DIC. High levels of elastase were observed in sepsis and APL. The levels of fibrinogen and fibrin degradation products were significantly higher in APL patients compared to patients with sepsis induced DIC. Nevertheless, the level of elastase degraded fibrin(ogen) was higher in the sepsis group (635.3 ng/ml, compared to 144.3 ng/ml in APL; p <0.0001). So, the enormous increase in fibrin and fibrinogen degradation products in APL cannot be explained by elastase activity. This study suggests a minor role for elastase mediated proteolysis in the hemorrhagic diathesis in APL patients.
急性早幼粒细胞白血病(APL)的出血综合征较为复杂,包括弥散性血管内凝血(DIC)和纤维蛋白溶解亢进。源自恶性早幼粒细胞的弹性蛋白酶被认为通过非特异性蛋白水解作用介导纤维蛋白原生成和纤维蛋白溶解。在本研究中,我们通过一种检测弹性蛋白酶降解纤维蛋白(原)的方法,测定了15例APL患者中弹性蛋白酶的作用,并将结果与脓毒症诱导的DIC患者的结果进行了比较。在脓毒症和APL患者中均观察到高水平的弹性蛋白酶。与脓毒症诱导的DIC患者相比,APL患者的纤维蛋白原和纤维蛋白降解产物水平显著更高。然而,脓毒症组中弹性蛋白酶降解纤维蛋白(原)的水平更高(635.3 ng/ml,而APL患者为144.3 ng/ml;p<0.0001)。因此,APL中纤维蛋白和纤维蛋白原降解产物的大量增加不能用弹性蛋白酶活性来解释。本研究表明弹性蛋白酶介导的蛋白水解在APL患者的出血素质中作用较小。