ADAMTS13

Typically, you most want to tell this apart from TTP or HUS.

And for that, you might need to test for ADAMTS13 enzyme deficiencies, because this is what's really going to differentiate it.

And if you treat one as the other, it's not going to work very well.

The treatments are very different.

If left unchecked, these massive multimers would grab too many platelets and cause runaway spontaneous clotting throughout the body

To prevent this, a specific plasma enzyme called ADAMTS-13 acts like molecular scissors

Continuously chopping the ultra-large VWF into safer, smaller functional pieces

And to ensure the clot only forms exactly where it is needed and doesn't propagate to block the entire vessel, healthy endothelial cells actively pump out powerful inhibitors