Törnebohm E, Egberg N, Sablica H, Wallin R, Lockner D, Paul C
Dept. of Internal Medicine, Huddinge University Hospital, Sweden.
Eur J Haematol. 1992 Aug;49(2):98-104. doi: 10.1111/j.1600-0609.1992.tb00038.x.
Proteolysis of coagulation factors and inhibitors resulting in haemorrhage can be mediated by elastase. Indirect signs of this are elevated levels of elastase complexed to its inhibitor in plasma, alpha 1-antitrypsin (E alpha 1-AT). We have measured intracellular elastase activity in leukaemic cells from 60 patients with acute leukaemia. Elastase activity was detected in 92% of the patients with acute nonlymphoblastic leukaemia (ANL), no activity was found in the patients with acute lymphoblastic leukaemia (ALL). High levels were found in cells from patients with promyelocytic leukaemia. Moderate to high total circulating blast elastase activity was measured in 70% of the ANL patients with haemorrhage compared with 36% of the patients without bleeding complications (p < 0.05). The available intracellular elastase activity was correlated to the level of E alpha 1-AT (rs = 0.42, p < 0.01) but not to the elastase specific split product of fibrinogen, B beta 30-43. In complete remission the levels of E alpha 1-AT were normalized. Intracellular elastase activity might be a useful supplement to differentiate ANL and ALL.
导致出血的凝血因子和抑制剂的蛋白水解可由弹性蛋白酶介导。其间接迹象是血浆中与抑制剂α1-抗胰蛋白酶（Eα1-AT）复合的弹性蛋白酶水平升高。我们测定了60例急性白血病患者白血病细胞内的弹性蛋白酶活性。在92%的急性非淋巴细胞白血病（ANL）患者中检测到弹性蛋白酶活性，而急性淋巴细胞白血病（ALL）患者未发现活性。早幼粒细胞白血病患者的细胞中发现了高水平的弹性蛋白酶。在70%有出血症状的ANL患者中检测到中度至高度的循环原始细胞弹性蛋白酶活性，而无出血并发症的患者中这一比例为36%（p<0.05）。细胞内可用的弹性蛋白酶活性与Eα1-AT水平相关（rs=0.42，p<0.01），但与纤维蛋白原的弹性蛋白酶特异性裂解产物Bβ30-43无关。完全缓解时，Eα1-AT水平恢复正常。细胞内弹性蛋白酶活性可能是区分ANL和ALL的有用补充。
