19 July 2011 (Tuesday) - ADAMTS13

I was going to witter on about thrombotic thrombocytopenic purpura today seeing how we had a case recently. But on doing a little research on the subject, I kept finding reference after reference to something called ADAMTS 13.
That was new to me – it turns out that ADAMTS13 is a zinc-containing metalloprotease enzyme that cleaves von Willebrand factor and degrades large vWf multimers, thus decreasing their activity.

Deficiency of ADAMTS13 has been described in various forms of TTP – reduced levels of this enzyme (acquired or genetic) causes large multimers of vWF to accumulate, activating platelets and causing microvascular clotting.

I wonder how long it will be before ADAMTS13 assays will be available on our coagulometers? Perhaps I had better start working on it...

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