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Hello, my name is Ruben Mesa,
and I´m the Executive Director
of the Mays Cancer Center
at UT Health San Antonio.
I'm delighted today
to present this
update on myelofibrosis
diagnosis,
pathogenesis, treatment and
issues with the survivorship
of these patients.
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Here are my disclosures.
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The myeloproliferative
neoplasms (MPNs) are a group of
chronic leukemias which
include myelofibrosis (MF),
polycythemia vera (PV), and
essential thrombocytopenia (ET).
They're heterogeneous and
they impact the
hematopoietic system.
Roughly in the United States,
we estimate about 350,000
patients with myelofibrosis
being the least prevalent.
We presenting symptoms can
be somewhat nonspecific,
often making the
diagnosis challenging.
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Natural history of the MPNs
begins with earlier disease
and by earlier disease
polycythemia vera,
essential thrombocytopenia
and what we call
early myelofibrosis or is now
termed pre-fibrotic
myelofibrosis.
In this phase of these diseases,
the time question
can be very latent.
There can be risked
vascular events.
There can be symptoms frequently
that can be associated
with the high counts and
difficulties with circulation.
Now, over time,
these diseases can
progress to myelofibrosis
and there's progression
is an important transition.
We normally feel that patients
with ET / PV and
early myelofibrosis
do not have
a dramatically different
life expectancy
than age match controls.
Although there certainly
are exceptions
in patients with
PV and early MF.
Those with overt
MF have much more
challenging phenotype
of the disease.
We have progressive
constitutional symptoms.
They may have
cytopenias that can
have significant enlargement
of the spleen or liver,
they may transform
to acute leukemia,
and the disease can
be life-threatening.
Roughly the life expectancy
in this phase of
the disease is three
to five years.
However, this can be
quite heterogeneous.
There are some very
long-lived individuals.
Additionally, many
of the therapies
which are in development,
may have an impact that may
help to prolong their survival.
