Mingers A M, Heimburger N, Zeitler P, Kreth H W, Schuster V
Children's Hospital, University of Würzburg, Germany.
Semin Thromb Hemost. 1997;23(3):259-69. doi: 10.1055/s-2007-996099.
Homozygous type I plasminogen (Plg) deficiency has not been described in human subjects so far. Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis of unknown etiology. Here we report for the first time on homozygous type I Plg deficiency in three unrelated female patients who suffered from ligneous conjunctivitis and additional pseudomembranous lesions of other mucous membranes. The disease is caused by massive fibrin depositions within the "extravascular space" of mucous membranes because of absent clearance by plasmin. Infusions of albumin, fresh frozen plasma, or Lys-plasminogen (Lys-Plg) into two of the three patients revealed normal Plg activation capacity in these patients. The absence of fibrinolytic activity could therefore be shown to be due to Plg deficiency. Similar studies in the third patient have not been completed. In the two patients studied so far, infusions of Lys-Plg resulted in prompt and adequate Plg recovery with a short half-life and high amounts of plasmin-antiplasmin complexes and D-dimer. One patient additionally revealed an inherited partial factor XII deficiency. Functionally, this factor XII deficiency did not interfere with Plg activation. However, there may be a pathway of Plg activation in this patient via the prekallikrein C1-INH system.
迄今为止，尚未在人类受试者中描述过纯合子I型纤溶酶原（Plg）缺乏症。木质化结膜炎是一种病因不明的罕见且特殊形式的慢性假膜性结膜炎。在此，我们首次报告3例患有木质化结膜炎及其他黏膜假膜性病变的无关女性患者存在纯合子I型Plg缺乏症。该疾病是由于纤溶酶清除功能缺失，导致黏膜“血管外间隙”内大量纤维蛋白沉积所致。对3例患者中的2例输注白蛋白、新鲜冰冻血浆或赖氨酸纤溶酶原（Lys-Plg）后，显示这些患者的Plg激活能力正常。因此，可证明纤溶活性缺乏是由于Plg缺乏所致。对第3例患者尚未完成类似研究。在目前已研究的2例患者中，输注Lys-Plg后可迅速且充分地恢复Plg水平，其半衰期短，并产生大量纤溶酶-抗纤溶酶复合物和D-二聚体。其中1例患者还存在遗传性部分因子XII缺乏。从功能上看，该因子XII缺乏并不干扰Plg激活。然而，在该患者中可能存在一条通过前激肽释放酶C1-INH系统激活Plg的途径。
