Week 11 Transcript Patho
Aplastic Anemia
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
Aplastic Anemia
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
