00:02
Finally, let's finish with another
important hematological disease,
although not a malignant disorder.
00:10
This is aplastic anemia.
00:13
And this is a rare condition.
00:15
But it's characterized
by low blood counts
due to the fact that
there's a reduction in cellularity
of the bone marrow.
00:24
Just look at that
slide on the right,
that is a bone marrow biopsy,
a terrifying biopsy
of the bone marrow.
00:33
And you can see there are
too many holes in that,
too many fat cells,
not enough hemopoiesis.
00:43
Now, why would this arise?
Well, in adults,
a probably arises we think,
because the immune system is
somehow damaging the stem cells
within the bone marrow
and reducing their numbers.
00:58
Just to get our terms correct,
bone marrow may be
actually hypoplastic
that means a reduced cellularity
or completely aplastic empty.
01:12
Out of interest,
that slide on the right
I would call hypoplastic
and you can see there is still
some hematopoiesis there,
but it's reduced from
what it normally would be.
01:23
Now then, let's think about
why aplastic anemia may arise.
01:30
Now, occasionally,
we see this in children.
01:34
And this usually arises because
of inherited abnormalities
that they've got due to damage genes
from their mother, or father.
01:42
Fanconi Anemia,
is one of the most common of these
collectively rare disorders.
01:49
And you'll see, on the top right,
some of the skeletal abnormalities
in the digits that you can see
in Fanconi Anemia.
01:58
And below that some of the
otter skin pigmentation
that you may also see here.
02:05
But in adults, aplastic anemia
usually arises out of the blue,
as we call idiopathic.
No obvious reason why.
02:15
Although it may occasionally
follow episodes of hepatitis
or an unusual reaction
to drug therapy.
02:24
We make the diagnosis by doing that
all important bone marrow trephine,
which shows the empty bone marrow
lacking in stem cells.
02:36
Then what can we do about it?
Well, treatment has
two major components.
02:43
One is supportive therapy.
02:45
We keep the patient alive despite
the fact that their bone marrow
is not working properly.
02:50
We can transfuse red cells.
We can transfuse platelets.
02:56
Neutrophils are more challenging,
and we don't usually do that
and we try to control infection with
antibiotics and antifungal agents.
03:07
A specific treatment aims to
reverse the process of aplasia
and start the bone marrow
being produced again.
03:17
And here we tend to use
immune suppression
with a drug is called
anti-lymphocyte globulin.
03:25
This is an unusual product in which
human lymphocytes are
injected into an animal,
perhaps a horse, or rabbit even.
03:34
And those animals
will make antibodies
against the human lymphocytes,
which we can purify
and give to patients.
03:41
And you can imagine
that that reduces the
lymphocytes in the patient,
and it's quite an
immune suppressive therapy.
03:50
It's been proven to be
pretty highly effective in patients
with aplastic anemia.
03:56
We often combine this
with a drug called cyclosporine.
04:00
That's an immune suppressant
drug as well,
and is often used in people
who have had a kidney transplant
to reduce organ rejection.
04:10
In patients with moderate disease,
that would be the approach
you would take
if they had a very
severe aplastic anemia
with very low blood counts.
04:19
You may also think of a
bone marrow transplant
an allogeneic bone marrow
transplant from another person
and that can be highly effective
in people with severe disease.
04:34
So in summary.
04:36
What we've seen in this lecture
is that a wide variety
of disorders may develop
within the hemopoietic system.
04:43
Myelodysplasia results
from a wide range of mutations
that damage the ability
of hematopoietic stem cells
to produce functional
and effective blood cells.
04:55
Myeloproliferative diseases have
a well defined genetic basis
and lead to over active bone marrow,
often with clinical splenomegaly.
05:05
And myeloma is a
malignant disease of plasma cells
leads to bone damage,
and paraprotein
which itself can cause a range
of clinical problems.
05:15
Finally, aplastic anemia is a rare
but serious condition
that may result from
stem-cell failure
perhaps as a result of
an inherited abnormality
or from autoimmune damage.
05:30
Thanks for watching this lecture
on hemopoietic malignancy.