Finally, let us finish with another important
haematological disease although not a malignant
disorder. This is aplastic anaemia. This is
a rare condition, but it is characterized
by low blood counts due to the fact that there
is a reduction in the cellularity of the bone marrow.
Just look at that slide on the right. That is a bone
marrow biopsy, a trephine biopsy
of the bone marrow and you can see there are
too many holes in that, too many fat cells
not enough haemopoiesis. Now, why would this
arise? Well in adults, it probably arises.
We think because the immune system is somehow
damaging the stem cells within the bone marrow
and reducing their numbers. Just to get all
terms correct, bone marrow may be actually
hypoplastic that means a reduced cellularity
or completely aplastic empty. Out of interest
that slide on the right, I would call hypoplastic
and you can see there is still some haemopoiesis
there but is reduced from what normally it
would be. Let us think about why aplastic
anaemia may arise. Now occasionally we see
this in children and this usually arises because
of inherited abnormalities that they have got
due to damaged genes in their mother or father.
Fanconi anaemia is one of the most common
of these collectively rare disorders
and you will see on the top right some of
the skeletal abnormalities in the digits which
you can see in Fanconi anaemia and below that
some of the. . . skin pigmentation which you
may also see here. But in adults, aplastic
anaemia usually arises out of the blue and
we call idiopathic. No obvious reason why.
Although it may occasionally follow episodes
of hepatitis or an unusual reaction to drug
therapy. We make the diagnosis by doing that
all important bone marrow trephine which shows
the empty bone marrow lacking in stem cells.
Then what can we do about it? The treatment has
two major components. One is supportive therapy.
We keep the patient alive despite the fact
that bone marrow is not working properly.
We can transfuse red cells. We can transfuse
platelets. Neutrophils are more challenging
and we do not usually do that and we tried to control
infection with antibodies and antifungal agents.
The specific treatment aims to reverse the
process of aplasia and start the bone
marrow being produced again and here we tend
to use immune suppression with the drug as
called anti-lymphocyte globulin. This is an
unusual product in which human lymphocytes
are injected into an animal perhaps the a
or rabbit even and those animals will make
antibodies against the human lymphocytes,
which we can purify and give to patients and
you can imagine that reduces the lymphocytes
in the patient and is quite an immune suppressive
therapy. It has been proven to be pretty highly
effective in patients with aplastic anaemia.
We often combine this a drug called cyclosporin.
That is an immune suppression drug as well
and is often used in people who had a kidney transplant
to reduce organ rejection. In patients with
moderate disease that would be the approach,
she would take if they have a very severe
aplastic anaemia with very low blood counts.
You may also think of a bone marrow transplant
and allogeneic bone marrow transplant from
another person and that can be highly effective
in people with severe disease. So In summary, what
we have seen in this lecture
is that a wide variety of disorders may develop
within the haemopoietic system. Myelodysplasia
results from a wide range of mutations that
damage the ability of haemopoietic stem cells
to produce functional and effective blood
cells. The myeloproliferative disease has
a well-defined genetic basis and leads to
overactive bone marrow often with clinical
splenomegaly. A myeloma is a malignant disease
of plasma cells leads to bone damage and a
paraprotein, which itself can cause a range
of clinical problems. Finally, aplastic anaemia
is a rare, but serious condition that may
result from stem cell failure perhaps as a
result of an inherited abnormality or from
Thanks for watching this lecture on hemopoietic malignancy.