Please wait while the transcript is being prepared...
0:00
Diagnosis and management of
hereditary spherocytosis
and elliptocytosis.
I am Dr. Vives Corrons.
I'm an Emeritus Professor at
the University of Barcelona.
I am working in the
Red Blood Cell and
Hematopoietic Disorders Unit of
the Institute for Leukemia
Research Josep Carreras
that is an important
institution in
Barcelona from our country,
Catalonia, in Spain.
0:28
The red blood cell
membrane plays
a crucial critical role
in the function and structure
of red blood cells.
It is a key determinant of
the unique disk
shape and provides
the cell with the finely
tuned combination
of flexibility and durability.
These are very
important properties
because this provides to red
blood cell the capability
to undergo an extensive
and repeated distortion
when the cell is circulated
in the blood stream
and also when it passes through
the microcirculation and
the spleen, especially
the spleen.
This allows to the red
blood cell to maintain
its normal life of
about 120 days.
Abnormalities of the red
blood cells membrane alter
or deform or produce
an abnormality on
the red blood cells
shape and compromise
this circulation of the
cell through the tissues,
especially in the
microcirculation
and the spleen,
creating a problem.
That means that the
cells are retained
locally and, at the end, are
destroyed and disappear.
This is called hemolysis,
and this is the cause of
the hemolytic anemia.
Hemolytic anemia is
characterized by a decrease of
the mean cell life
that I mentioned
before, the 120 days.
So, it becomes about 10, 20, 50,
or 60 days, very lower
than the normal value.
Well, in fact,
there are two examples of
the most frequent red blood cell
membrane disorders, that are
also the best work known,
is the hereditary spherocytosis,
in which the red blood
cells become spheric,
and the hereditary
elliptocytosis,
in which the cell becomes
elongated or elliptical.
However, I will also
mention a very rare
red blood cell
membrane syndromes, we have
called syndromes that are
characterized by an abnormal
membrane permeability
to red cell cations,
and you will cause
either a hydration,
an overhydration of the red
blood cell or a dehydration.
These are the
disorders known with
the names of stomatocytosis
overhydration,
or xerocytosis dehydration
of the red blood cells.
