Leebeek F W, Knot E A, Ten Cate J W, Traas D W
Department of Internal Medicine II, University Hospital Dijkzigt, Rotterdam, The Netherlands.
Am J Hematol. 1989 Jan;30(1):32-5. doi: 10.1002/ajh.2830300107.
We report a 45-year-old female patient with recurrent spontaneous deep vein thrombosis associated with an isolated hypoplasminogenemia (plasminogen activity and antigen level of 42% and 37%, respectively). The plasminogen molecule was normal as demonstrated by a normal activation by tissue plasminogen activator, electrophoretic mobility on crossed immunoelectrophoresis, molecular weight, and binding to lysine sepharose. All other hemostatic parameters predisposing to recurrent thrombosis were normal. A stimulation test with desmopressin acetate (DDAVP) showed a normal plasma rise of both tissue plasminogen activator and factor VIIIR:WF. This isolated plasminogen deficiency apparently is due to a decreased synthesis of a normal plasminogen molecule and is associated with a severe thrombotic tendency.
我们报告了一名45岁的女性患者,患有复发性自发性深静脉血栓形成,伴有孤立性纤溶酶原血症(纤溶酶原活性和抗原水平分别为42%和37%)。组织纤溶酶原激活剂的正常激活、交叉免疫电泳的电泳迁移率、分子量以及与赖氨酸琼脂糖的结合均表明纤溶酶原分子正常。所有其他易导致复发性血栓形成的止血参数均正常。醋酸去氨加压素(DDAVP)刺激试验显示组织纤溶酶原激活剂和因子VIIIR:WF的血浆水平均正常升高。这种孤立性纤溶酶原缺乏显然是由于正常纤溶酶原分子合成减少所致,并与严重的血栓形成倾向相关。