Weinger R S, Rudy C, Moake J L, Olson J D, Cimo P L
Blood. 1980 May;55(5):811-6.
A 72-yr-old male with a lifelong history of easy bruisability and posttraumatic bleeding had a prolonged prothrombin time and activated partial thromboplastin time. His plasma Stypven, Taipan, and Echis carinatus venom clotting times were prolonged. The presence of a dysprothrombin was confirmed by the discrepancy between plasma prothrombin coagulant activity and prothrombin antigen levels. His plasma prothrombin was capable of being completely absorbed onto and then eluted from barium sulfate. Crossed immunoelectrophoresis of his plasma prothrombin, and normal plasma prothrombin, into agarose containing rabbit anti-human factor II antibody were similar. Crossed immunoelectrofocusing, a procedure combining isoelectric focusing in disc gels with electroimmunoassay in the second dimension, demonstrated that the patient's prothrombin antigen was more basic than normal. The eluate from barium sulfate absorbtion of patient plasma, when reacted with Echis carinatus venom (which directly cleaves prothrombin to thrombin) clotted purified fibrinogen at a rate slower than normal plasma eluate. SDS-slab gel electrophoresis revealed that the prothrombin present in the patient's eluate was cleaved by Echis carinatus venom. These studies suggest that the coagulopathy of prothrombin Houston results from the generation of a dysfunctional thrombin.
一名72岁男性，有终身易瘀斑和创伤后出血病史，其凝血酶原时间和活化部分凝血活酶时间延长。他的血浆罗素蝰蛇毒、大班蛇毒和锯鳞蝰蛇毒凝血时间延长。血浆凝血酶原凝血活性与凝血酶原抗原水平之间的差异证实了异常凝血酶原的存在。他的血浆凝血酶原能够完全吸附到硫酸钡上，然后从硫酸钡上洗脱下来。将他的血浆凝血酶原和正常血浆凝血酶在含有兔抗人因子II抗体的琼脂糖中进行交叉免疫电泳，结果相似。交叉免疫电聚焦是一种将圆盘凝胶中的等电聚焦与二维免疫电泳相结合的方法，结果表明患者的凝血酶原抗原比正常情况更具碱性。患者血浆经硫酸钡吸附后的洗脱液与锯鳞蝰蛇毒（直接将凝血酶原裂解为凝血酶）反应时，使纯化纤维蛋白原凝固的速度比正常血浆洗脱液慢。SDS平板凝胶电泳显示患者洗脱液中的凝血酶原被锯鳞蝰蛇毒裂解。这些研究表明，凝血酶原休斯顿型凝血障碍是由功能失调的凝血酶生成所致。
