Thrombotic Thrombocytopenic Purpura (TTP) is a hematologic emergency characterized by fever, altered mental status, thrombocytopenia, acute kidney injury, and a characteristic rash, caused by a deficiency in ADAMTS13 enzyme that normally cleaves von Willebrand factor into smaller multimers; without this cleavage, large vWF multimers cause platelet clumping, microthrombus formation, and hemolysis leading to end-organ damage.
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Mark Ramzy, DO (@mramzydo) from ResusX:ReUnion talks recognizing and treating TTP. #shorts本站添加:
This is a relatively young male who has a fever, altered, thrombocytopenic, acute kidney injury, and that rash.
Well, if you said TTP, you are correct.
Thrombotic thrombocytopenia purpura. And again, that's a mouthful, so we're going to say TTP. Well, this is an inherited or acquired deficiency of the ADAMTS13 gene. So, what happens here is you have von Willebrand factor, and ADAMTS13 gene cleaves von Willebrand factor into smaller multimers. Now, those smaller multimers can actually diffuse throughout the rest of the body and can cause some platelet clumping, microthrombus formation, and hemolysis. And in severe cases, pretty significant organ dysfunction.
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