Let’s take a look at paroxysmal
Everything that you want to know about
this condition is actually in the name.
And so let’s go ahead and
dissect each part of this.
First, my issue is the fact that,
well, I’m not able to properly
control the destruction of my RBC.
Now, a couple of other things as well that
you want to keep in mind, remember earlier,
we had our discussion of G6PD deficiency
and I told you to keep it as a differential,
chronic granulomatous disease.
Well, here, with this picture
of paroxysmal nocturnal,
granted we’re going to
focus upon the RBC.
However, as far as you’re concerned,
the clinical picture
is a lot broader.
What I mean by that is if you take
a look at the statement here,
you’ll understand that not
only is my RBC being affected,
as is my neutrophil, but you
really, ladies and gentleman,
the clinical picture of
platelets becomes important.
What I mean by that is it's
not that you’re going to have
of your platelet.
In fact, you end up having excess
stimulation of platelet function.
So if you have excess
platelet functioning or
you have excess activity of your
platelets, then you’re forming tons of?
Up and down the body.
That’s your first clinical picture.
The patients that walk in with PNH are suffering
from some kind of thrombotic episode,
Well, maybe there might be
Well, the portal vein underwent
So imagine now, the portal vein,
which should normally be draining
your superior mesenteric vein and the
splenic vein have now become thrombosed.
And if it’s thrombosed,
then you’re going to back up into,
well, your portal hypertension.
So that will be your esophageal
varices, that will be caput medusae,
and that will be your
hemorrhoids, wouldn’t it?
And so that’s portal vein thrombosis.
What if it was an issue
with my hepatic vein?
You’d call that Budd-Chiari, don’t you?
So Budd-Chiari type of syndrome
would be an issue in the liver,
especially zone 3 or there might
be inferior vena cava thrombosis,
you get my point.
So you want to be very smart about
how you are developing the thrombus,
where you’re developing
and how your patient’s going
to present with that thrombus.
Platelets is an issue.
What about these RBCs?
Take a look at the name.
Nocturnal, most commonly at night.
And then you wake in the morning and
you find red urine, hemoglobinuria.
At this point, you should be really
familiar with the term hemoglobinuria
and understanding what kind
of hemolysis would this be.
Intravascular hemolysis, what
happens to your haptoglobin level?
Remember hemoglobin requires a chaperone.
It binds to haptoglobin,
free levels of
haptoglobin will drop.
This complex will then
go to your glomerulus
and the hemoglobin will then get
filtered, resulting in hemoglobinuria.
Well, one of the discussions that we had
earlier when we did microcytic anemia,
was that when you are losing hemoglobin,
along with it what might you be losing?
What is that heme comprised of?
Oh, yeah, iron.
So during hemoglobinuria,
you might also be losing iron
resulting in iron deficiency anemia.
A lot of things here of
this particular statement,
but I’m giving you a global picture of
paroxysmal nocturnal hemoglobinuria.
We will then walk into further details.
Now, there is a specific
component of your deficiency
and this is known as a
Keep that in mind because at some point
you will be responsible in great depth
to go into what’s known as flow cytometry
with PNH, GPI-linked proteins.