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Hello everyone. I'm
Pratima Chowdary.
I'm a Hematologist at the
Royal Free Hospital in London.
Today I will be providing
an overview of the use of
non-factor therapies
for the management
of haemophilia A and B.
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These are my disclosures.
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The haemophilias are
the most common X-linked
inherited monogenic
bleeding disorders.
In patients with haemophilia A,
we have abnormalities of
Factor 8 gene which results
in deficiency of Factor VIII.
The incidence is about
one in 5,000 males.
Similarly in haemophilia B,
we see abnormalities
of the Factor 9 gene,
which results in deficiency
of Factor IX and
the worldwide incidence is about
one in 30,000 male infants.
The normal levels of
Factor VIII and IX are
between 50 and 150
IU per deciliter.
The severity of the deficiency
determines the
clinical presentation.
In patients with severe
haemophilia A or B,
the factor levels are
less than one percent.
This typically manifests
as spontaneous bleeding
into joints or
muscles in the absence of
any obvious
hemostatic challenge.
That means there's no trauma or
surgery that is
triggering bleeding.
The patients can also bleed
into any tissue or organ.
We also see bleeding
after trauma and surgery.
In patients with
moderate haemophilia,
the levels are between
one and five percent
and spontaneous bleed
is not as common as in
patients with severe disease.
Patients with moderate
disease do have
prolonged bleeding with
minor trauma or surgery.
In patients with
mild haemophilia,
the levels typically tend to be
more than five percent
and under 40 percent.
Spontaneous bleeding is unusual
in this group of patients.
But we can see
prolonged bleeding
with major trauma or surgery.
