Pechet L, Snyder L M, Chesney C, Colman R W
Thromb Diath Haemorrh. 1974 Sep 30;32(1):90-104.
An 85 year old woman was studied because of severe bleeding. Acquired inhibitors to factor VIII and to phospholipid procoagulants were demonstrated. Platelet factor 3 (Pf3) assay was prolonged with both kaolin and Russell's Viper Venom (Stypven-R). It was normal with patient's washed platelets and normal plasma, but abnormal when normal platelets were incubated with patient's plasma. The inhibitor also blocked the coagulant action of Bell and Alton thromboplastin, inosithin, phosphatidyl ethanolamine and phosphatidyl choline, but not that of tissue thromboplastin or cardiolipin. All other platelet functions were normal. The inhibitors were purified by Al (OH3) absorption, heating at 56degrees, precipitation by 50% ammonium sulfate, followed by dialysis and DEAE-cellulose chromatography. A partial separation of the two inhibitors was achieved. Cyclophosphamide treatment resulted in cessation of bleeding and dissappearance of the inhibitors. This seems to be the first instance of an acquired circulating inhibitor specifically directed against phospholipid procoagulants in a patient who also had an inhibitor to Factor VIII.
一名85岁女性因严重出血接受检查。检测发现存在针对凝血因子VIII和磷脂促凝剂的获得性抑制剂。高岭土和罗素蝰蛇毒（Stypven - R）检测时血小板第3因子（Pf3）检测时间延长。患者洗涤血小板与正常血浆检测时结果正常，但正常血小板与患者血浆孵育时结果异常。该抑制剂还可阻断贝尔和奥尔顿凝血活酶、肌醇磷脂、磷脂酰乙醇胺和磷脂酰胆碱的促凝作用，但不影响组织凝血活酶或心磷脂的促凝作用。所有其他血小板功能均正常。通过氢氧化铝吸附、56℃加热、50%硫酸铵沉淀、随后透析和DEAE - 纤维素色谱法对抑制剂进行纯化。实现了两种抑制剂的部分分离。环磷酰胺治疗使出血停止且抑制剂消失。这似乎是首例在患有凝血因子VIII抑制剂的患者中出现针对磷脂促凝剂的获得性循环抑制剂的病例。
