Prothrombin Houston: a dysprothrombin identifiable by crossed
immunoelectrofocusing and abnormal Echis carinatus venom activation
RS Weinger, C Rudy, JL Moake, JD Olson and PL Cimo
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.
Volume 55,
Issue 5,
pp. 811-816,
05/01/1980
Copyright © 1980 by The American Society of Hematology
