All right, so welcome back to the Internet Book of Critical Care podcast.

I'm here with Adam Thomas and we're going to talk about acute pro myocytic leukemia.

Josh, this is one of those hematologic oncologic emergencies that we need to get right

at admission because we need to start this therapy right away and recognize it.

And there's not a lot of those disorders in this section of the textbook are there?

And even within the leukemia range, and that's like a bad collection of disorders to begin with,

this is uniquely bad.

So here we'll talk about why APL is really important to recognize.

We'll talk about the specifics around differentiation syndrome, other things

around treatment, and then re-emphasize over and over and over again how important it is to

recognize the DIC associated with this type of leukemia. So Josh, start it off. Let's get straight

into the path of physiology. The molecular biology here is a little painful, but I actually think

it's pretty fascinating,

and it explains really everything that's going on.

So it's worth a couple minutes on.

So basically, this malignancy occurs when there's a translocation, two chromosomes kind of get

stuck to each other that should not be stuck to each other that generates a fusion protein

between PML and this rarogen.

The rarer gene is a retinocyan acid receptor. And the fusion protein on pro myocytes prevents them from differentiating into myocytes.

And that causes the pro mylilocytes to become immortal and just continue replicating

indefinitely leading to malignancy.

And that's what causes leukemia.

...