Yusuf

Fibrinogen level was normal.

The mixing study was done, and there was no correction with mixing.

The von Willebrand's factor level was within normal limits.

There was a normal ristocetin cofactor activity that Mark wanted in aliquot one.

And all the other coagulation factor levels were within normal limits.

And I'm going to pause here for like final discussion or what we do all the next.

Yeah, yeah, I'm excited to give you all some more information.

So in this case, he did end up getting a pan scan that was normal, and S-PEP was normal.

And then they sent an inhibitor titer, which is the Bethesda inhibitor titer, and his titer was very high, basically diagnostic of a factor VIII inhibitor.

Okay.

So especially given his new spontaneous bleeding, very low factor level, very high inhibitor level, and normal von Willebrand's factor level, that's virtually a diagnostic of the disease.

So I have a few teaching points.

So I'm going to tell you what happened to this patient.

So this patient was actually treated with a recombinant factor VIII and then cyclophosphamide

rituximab, and dexamethasone.

The patient underwent a graft, skin graft, and then actually did pretty well and was discharged from the hospital to a subacute rehab and was able to follow up our patient.

And I have a few teaching points.

There's a NEJM paper from 2016 on this topic that this first line is iconic, so I'm going to repeat it here.

Heme modules in medical schools teach about TTP and AML as the heme emergencies.

Factor inhibitors should be added to that list.