So now, there are membrane
fragments that are being lost.
You are then going to lose that biconcave
shape and you become a sphere.
Now, that you have
a misshapen RBC,
do you remember that
picture in our discussion
where we’re talking
about a spherocyte?
And inside the spleen,
you had these splenic macrophages and
remember your guests were saying,
“Oh, you really shouldn’t have brought
us a spherocyte because we just love it."
And so you have a splenic macrophage
that is just going to go crazy.
And eat these spherocytes.
So therefore, what kind
of hemolysis is this?
So therefore, you’re patient is
going to have which one of these?
or significant jaundice?
let’s take a look at
what we have here.
Mutations in ankyrin or spectrin, I do
want you to know two membrane components.
The first one that I showed
you especially was spectrin,
but think of this
as being an anchor.
There’s a protein
component called ankyrin.
And if this also becomes lost, then you
have as you seen in the picture here,
a perfectly, perfectly formed spherocyte
in which the central
pallor has been lost.
there are some RBCs in this picture
in which there are central pallor,
okay, so you will
have a mixed picture.
You can't say that “Oh well, the entire
picture is going to be all spherocyte."
That’s not practical.
But you have enough RBCs
here that you’re seeing
in which you should be able to come up
with a diagnosis of at least two actually.
Are you ready?
So two differentials.
“So Dr. Raj, you’re telling me that
there are two differentials or anemias
that then appear where the RBC
appears as being a spherocyte?”
The two major ones are going to be
either HS, hereditary spherocytosis,
the other one we’ll take a look at and
that you must be very familiar with
is the warm type of
autoimmune hemolytic anemia.
I will mention that once
again and again and again
until we get these
So how can you tell the
difference between the two?
Is this an autoimmune disease,
So therefore anything that you would do
in terms of your testing for immunity
or autoimmunity is something
called a Coombs test.
And that Coombs test is going to be
negative for hereditary spherocytosis.
Where the antihuman globulin test or
the Coombs test will be positive for
warm type of autoimmune
Keep that in mind as we move forward.
I cannot just give you something
and not have you realize
that students can get this
confused with other conditions.
Now, let’s now take a look at
the involvement of the spleen,
but then where am I?
This is the gallbladder.
So you connect the splenic destruction
of your bilirubin or your hemoglobin
to what we’re seeing
here in the gallbladder.
This is cholelithiasis,
the type of stones that
you’re seeing here,
don’t they appear rather
black and pigmented?
So it cannot be
So don’t ever choose that.
If it’s extravascular hemolysis, please,
this is pigment stones made up of
bilirubin so they’re black and pigmented.
This is a sign for
Now, in addition, what else may happen?
Well, you also have splenomegaly,
so you want to keep that in mid.
And an important test is this.
I asked you earlier about that osmotic
pressure within that concentrated RBC
that I showed you a
picture of earlier.
So if you have increased concentration of RBC
and you put this into a saline solution,
in other words, you’re putting this
into a more hypotonic solution,
then what can you imagine
and what can you think,
what’s the behavior of
this water going to do?
You can predict that the water is going
to go into the concentrated spherocyte.
What do you call this?
The osmotic fragility test.
So what is going to happen
to size of the cell?
It is then going to increase in size.
Now, that’s called the
osmotic fragility test
and with the spleen being enlarged,
you are thinking about doing what?
Maybe perhaps a splenectomy.
And whenever you are compromising
the function of the spleen,
what kind of organisms
are you worried about?
You’re worried about this encapsulated
organisms especially Streptococcus pneumoniae.
You also Haemophilus influenzae,
So you have a bunch there.
And then what we see here
is calcium bilirubinate
and this is increased conjugated
bilirubin in the bile.