Essential Thrombocythaemia - (ET) - A Guide for Patients - Leukaemia Care
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Essential Thrombocythaemia (ET) A Guide for Patients
Introduction
Being diagnosed with essential thrombocythaemia (ET)
can be upsetting, particularly when you may never have
heard of it before, and may even have had no obvious
symptoms. If you have questions about ET – what causes
it, who it affects, how it affects your body, what symptoms
to expect and likely treatments – this booklet covers the
basics for you.
You’ll also find useful advice reviewed by Manos Nikolousis,
about how to get the best from Consultant Haematologist at
your haematologist, plus practical Heart of England NHS Trust. The
advice on how to help important booklet has then been updated
people in your life understand by our Patient Information Writer,
such a rare condition. For Isabelle Leach, and reviewed by Dr.
more information, talk to your Mallika Sekhar, UCLH. We are also
haematologist or clinical nurse grateful to Linda Phyall, Sabine
specialist. Lurcook, Rachel Worzencraft,
Sue Law, Dr Dick Morris, Paula
This booklet was originally written
Davis, Jeanette Bennett, Fiona
and subsequently revised by Ken
Bridge, Claire Todd, Cath Owens
Campbell MSc (Clinical Oncology)
and Lucy Geering for their
and the review was conducted by
valuable contributions as patient
Lisa Lovelidge. It was then peer
reviewers.
If you would like any information on the sources
used for this booklet, please email
communications@leukaemiacare.org.uk
for a list of references.
Version 4
Printed: 07/2020
2 www.leukaemiacare.org.uk Review date: 07/2022In this booklet
Introduction 2
In this booklet 3
About Leukaemia Care 4
What is essential thrombocythaemia (ET)? 6
Symptoms of ET 8
How is ET diagnosed? 10
What is the prognosis of ET? 11
Treating ET 12
Living with ET 16
Talking about ET 20
Glossary 23
Useful contacts and further support 27
Helpline freephone 08088 010 444 3About Leukaemia Care
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Helpline freephone 08088 010 444 5What is essential
thrombocythaemia (ET)?
ET is a chronic condition Organisation who updated their
characterised by too many classification in 2016 to include
platelets in the blood. It belongs MPNs such as ET.
to a group of conditions called
Blood cells which are produced
myeloproliferative neoplasms
from stem cells in the bone
(MPNs), which also includes
marrow include:
polycythaemia vera (PV) and
myelofibrosis (MF). 1. Red blood cells (to carry oxygen
to the tissues of your body)
If you would like more 2. White blood cells (to fight
information about PV and infection and disease)
MF, you can download our
booklets from our website 3. Platelets (to help blood to clot)
at www.leukaemiacare.
Production of new blood cells is
org.uk or request a copy of
very closely controlled in the bone
the booklets by emailing
marrow so that it is balanced
support@leukaemiacare.
with the loss of worn-out cells or
org.uk or calling the
cells lost by bleeding or damage.
helpline on 08088 010
In people with ET, there is an
444.
excess of platelets. Although it
is platelets that are primarily
There has been some debate affected, white blood cell levels
about whether or not MPNs are may also be elevated.
types of cancer. This is because
the word neoplasm (which means In patients with ET, the blood
new growth) is a term used for becomes thicker than normal and
cancers (malignant neoplasms) the excess number of platelets
and noncancerous tumours may cause blood clots to form
(benign neoplasms). In ET there is more easily. Clots can block blood
an uncontrolled increase in cells; flow through veins and arteries,
therefore, many haematologists potentially leading to heart
and cancer organisations do attacks or strokes.
consider MPNs as blood cancers.
ET is also associated with an
This includes the World Health
increased risk of bleeding
6 www.leukaemiacare.org.ukcomplications. your lifetime and is not present at
birth.
In some cases, ET can transform
to MF or acute myeloid leukaemia In addition, a mutation in a gene
(AML). known as CALR is present in 15%
to 30% of patients with ET, and the
Who is affected by ET? MPL mutation is seen in 4% to 8%
of patients.
ET is considered to be a rare
disease. The number of people Some 10% to 20% of patients with
diagnosed with ET in Europe is 0.6 ET do not express any of these
per 100,000 patients per year. This three mutations. This is referred
may explain why you might not to as triple-negative.
have heard of ET or met anyone
with the condition before. It is also important to be aware
that, although some families
ET is most common in those aged seem to develop the disease
over 60 years. more readily than others, ET is
usually not inherited nor passed
It is more common in women
on from parent to child. However,
than men, with two women
and this is less common, an
being diagnosed for every man,
increased likelihood of the gene
particularly in patients who
mutating (which can then lead to
are less than 60 years of age.
developing ET) can be inherited
However, it must be noted that
and is called familial ET. It
20% of patients are older than 40
resembles non-familial ET both in
years of age at diagnosis.
terms of clinical symptoms and
What causes ET? mutations.
While the exact cause of ET is Finally, some researchers believe
not known, research has found MPNs may also be triggered
that approximately 55% of people by past exposure to ionising
who have ET have a mutation in radiation (a type of radiation
a protein that regulates blood that has very high energy, like
cell production. This protein is medical x-rays or nuclear fallout)
known as Janus Kinase 2 (JAK2) or to some chemical substances
and the mutation itself is JAK2 such as benzene and toluene.
V617F. This mutation arises during
Helpline freephone 08088 010 444 7Symptoms of ET
Many patients with ET do not • Reddish or purple skin
have any symptoms when they
are diagnosed. Typically, these • Bleeding or clotting
patients are identified following • Unexplained bruising
abnormal results during a routine
full blood test for something else. This is not an exhaustive list
If symptoms do develop, they tend of symptoms. There is a wide
to do so over time. variety of symptoms that can be
attributed to a diagnosis ET, so it
If you have persistent symptoms is important to make your doctor
of ET, make an appointment to aware of these. Not everyone will
see your GP. Common symptoms have the same symptoms or to
of ET include: the same degree of severity. It is
also important to understand that
• Fatigue the presence of these symptoms
• Night sweats does not always indicate ET
and they could be due to other
• Headaches (this can also conditions and illnesses.
include migraines with visual
disturbances) In patients with ET, the increased
numbers of platelets and white
• Itching blood cells can result in blood
• Fever clots (thrombosis) in a vein or
artery, or they may cause bleeding
• Swollen spleen (located under (haemorrhage).
the ribs on the left of the
abdomen, this organ helps to Blood clots or bleeding are a
rid the body of toxins, waste and major complication of ET. There
other unwanted materials) is a 1% to 3% risk of arterial or
venous blood clots occurring in
• Bone pain a patient per year; however, if the
• Weight loss patient has a JAK2 mutation, this
risk increases to 7.7%.
• Dizziness or light headedness The blood clots prevent the
8 www.leukaemiacare.org.ukblood from flowing and the most you will then have the functioning
common complications caused by of your platelets tested. Your
these blood clots are: haematologist will closely
monitor any antiplatelet drugs or
• Heart attacks, strokes or anticoagulant drugs you may be
damage to the gastro-intestinal
receiving. Any significant bleeding
tract due to blood clots in the
episodes will be managed with
arteries.
tranexamic acid, which stops
• Venous thrombosis such as bleeding in the short term, or a
deep vein thrombosis in the transfusion of platelets.
calf.
General risk factors that can
• Pulmonary embolisms, where a increase your likelihood of blood
clot in the vein travels through clots or bleeding include:
the blood stream and causes a
blockage in one of the arteries
• Being older than 60 years
in the lungs. • Previous blood clots or bleeding
The risks of bleeding are less • A high platelet count
clearly defined, mostly because
the definition of bleeding in • JAK2 mutations
various studies is different. • Having a cardiovascular risk
Risks of bleeding are known to factor, such as high blood
be increased if the platelet count pressure, diabetes, smoking or
is very high (between 1000x109/L high cholesterol
and 1500x109/L). It could also be
related to acquired von Willebrand Enlargement of the spleen is
syndrome with extreme levels of present in 10% to 20% of patients
platelets. with ET at diagnosis.
If you have experienced some
bleeding, you may be screened
for acquired von Willebrand
syndrome. If the test is negative,
Helpline freephone 08088 010 444 9How is ET diagnosed?
ET is often suspected if a routine tests including:
blood test shows that a patient
has a high platelet count, also • Blood tests – Blood tests can
identify an increase in blood
called thrombocytosis.
cells and exclude other causes
To make a diagnosis of ET, the of a high cell count.
following criteria must be present:
• Gene mutation analysis –
• A platelet count of 450x10 /L or
9
This is to identify any gene
higher mutations you may have,
particularly JAK2, CALR or MPL.
• A bone marrow biopsy
showing an increase in the • Bone marrow investigations
numbers of enlarged, mature – You may have either a bone
megakaryocytes (cells in the marrow aspiration or both an
bone marrow that produce aspiration and a bone marrow
platelets) biopsy. This will be done to look
for classic signs of ET. During
• Not meeting the diagnosis for a bone marrow aspiration, the
another MPN, such as PV or MF
doctor or nurse takes some
• The presence of a JAK2, CALR or bone marrow cells up into a
MPL mutation syringe. A bone marrow biopsy is
when they remove a one to two-
If none of the mutations are centimetre core of bone marrow
present, other causes of primary in one piece using a trephine
or secondary thrombocytosis (a surgical instrument with a
as well as diagnoses of PV and cylindrical blade). The sample is
MF must be excluded. Primary then sent to the laboratory for
refers to a condition that is the testing.
root cause of the illness, whereas
secondary refers to a condition
that has developed as a result of
another one.
ET is diagnosed using laboratory
10 www.leukaemiacare.org.ukWhat is the prognosis of ET?
Treatment is focussed mainly on or MPL does not affect the survival
preventing any complications of patients with ET. However, older
from blood clots or bleeding age, a high white blood cell count
which is associated with ET. Low- and a previous history of blood
risk patients are best managed clots and anaemia are associated
with low-dose aspirin or watch with a poor survival.
and wait alone, and patients with
In some patients, ET can develop
high-risk ET will benefit mainly
into MF or AML. However, this is
with cytoreductive therapy and
rare. Transformation to AML risk
anticoagulation.
factors include:
When properly monitored and
treated, patients with ET have • Advanced age
an excellent prognosis and life • Leucocytosis (a high white
expectancy. Your prognosis can blood cell count)
be influenced by a number of
factors including your individual • Exposure to previous
situation, your health history and treatments which suppress
the way you respond to treatment. the bone marrow, such as
ET is a chronic condition; chemotherapy
therefore, it is important for you The prognosis of patients
to have a positive partnership with ET can vary widely. Your
between your haematology team. haematologist is the best person
See your haematologist regularly to advise you based on your
and report any new or different individual circumstances.
symptoms.
For the majority of patients whose
ET does not progress to MF or
AML, a normal or very slightly
reduced life expectancy can be
anticipated.
Having the mutations JAK2, CALR
Helpline freephone 08088 010 444 11Treating ET
Overview of treatment blood clots.
Most treatments for ET are
intended to manage your
Medications
symptoms and prevent any Cytoreductive therapy
associated problems from blood
Cytoreductive therapy is
clots or bleeding in order to
important in enabling ET and its
maintain your quality of life.
complications to be controlled. It
Treatments for ET are aimed is the only treatment significantly
at lowering the production of associated with reducing the
platelets and maintaining normal occurrence of blood clots. In
blood counts. This is called patients with ET, first-line
cytoreductive therapy. There are a treatment is hydroxycarbamide,
number of medications which can with second-line treatment being
achieve this. anagrelide.
Treatment according to Hydroxycarbamide (also known
as hydroxyurea)
risk factors
This is the most commonly used
Treatment will be based on an
chemotherapy drug to treat ET
assessment of your risk factors
and is available as a tablet or
regarding blood clots and
capsule.
bleeding. While cytoreductive
therapy is fundamental in An early study of patients with
treating ET, managing any high-risk ET confirmed that
potential blood clots or bleeding the percentage of patients
is equally important. Your receiving hydroxycarbamide had
medical team will give you all the significantly less blood clots
information about the treatment or bleeding (3.6%) compared
which is best for you. with patients not receiving
any treatment (24%). In recent
Low-dose aspirin and
controlled studies of patients
anticoagulants may be used to
with high-risk ET, neither
prevent any blood clots, especially
anagrelide nor interferon alpha
if you have a history of developing
could be shown to be superior to
12 www.leukaemiacare.org.ukhydroxycarbamide for reducing developing into AML.
blood clots or bleeding.
Anagrelide
Hydroxycarbamide can cause
Taken as a capsule, anagrelide is a
mild side effects such as:
drug that prevents the maturation
• Increased risk of infection of platelets. It is used to counter
the overproduction of platelets. It
• Bruising or mild bleeding lowers the platelet count and has
some effect on the red blood cells.
• Anaemia (as it reduces all types
of blood cells, including red Anagrelide is approved for
blood cells) reducing elevated platelet counts
in at-risk patients with ET who are
• Fatigue intolerant to their current therapy
• Diarrhoea or constipation or whose elevated platelet counts
are not reduced to an acceptable
• Sore mouth level by their current therapy.
• Changes to the skin, including Side effects of anagrelide include:
ulceration and increased risk of
developing skin cancer • Headaches
Hydroxycarbamide may also • Diarrhoea
affect fertility. If you are taking
it, you will be advised not to get • Palpitations
pregnant or father a child, as • Fluid retention
there may be a risk of harming the
developing baby. It is advisable to Interferon alpha
use effective barrier contraception Interferon alpha is a substance
while taking the drug and also which occurs naturally in the body
for a few months afterwards. If and reduces the rate at which
hydroxycarbamide is used either blood cells, including platelets,
alone or in combination with are made in the bone marrow. It
other chemotherapy drugs over a can be made into a medicine to
long period of time, it may slightly be given as an injection under
increase the chance of the ET the skin to treat a wide range of
Helpline freephone 08088 010 444 13Treating ET (cont.)
conditions, including ET. scarring.
Side effects of interferon alpha New treatments
include:
• JAK2 inhibitors – JAK2
• Flu-like symptoms inhibitors block the function of
the JAK2 mutation which slows
• Headaches down blood cell production,
• Vision disturbances reduces spleen size and
improves symptoms. Up to 55%
• Depression of patients with ET have been
• Liver and thyroid disease found to have a JAK2 mutation.
JAK2 inhibitors are being looked
However, it does not increase the into as a treatment for ET.
risk of secondary leukaemia and
can be used in pregnancy. Prevention of blood
Busulfan
clots and bleeding
Busulfan is usually given to
Aspirin
patients who have side effects As well as being used for reducing
when taking hydroxycarbamide. pain and lowering temperature,
It can be given as a tablet. Like aspirin is known to prevent
hydroxycarbamide, busulfan platelets sticking together
affects the bone marrow directly and may reduce your risk of
and can lead to a fall in the blood developing a blood clot.
cell counts. Its main side effects
In patients with very low-risk
are nausea and low platelet
ET, aspirin might be required.
counts.
In patients with low-risk ET,
Your haematologist can advise treatment with low-dose aspirin is
you if busulfan is suitable, advised, especially if the patient
and will monitor your progress has cardiovascular risk factors, as
carefully during your treatment. it has been shown to reduce both
There is evidence that busulfan venous and arterial blood clots.
can increase the risk of secondary
Research shows that aspirin is
leukaemia and can cause lung
very effective at reducing risks of
14 www.leukaemiacare.org.ukheart attacks and strokes in many a venous blood clot that has
people with different levels of risk. occurred with no identifiable risk
Patients with intermediate-risk ET factor. This will be done after your
and high-risk ET will be given low- bleeding risk has been assessed.
dose aspirin whether they have
Patients with ET and other MPNs
cardiovascular risk factors or not.
more commonly have unusual
Low-dose aspirin can have some sites of venous clots, meaning
side effects such as bleeding and that the use of anticoagulation
indigestion. It can also cause treatment can be particularly
gastric irritation, bleeding in the beneficial for them. These unusual
stomach, and ulcers. You may sites of venous clots include:
find that you bruise more easily
and that you bleed for a long • The splanchnic vein – this
drains the blood from the
time if you cut yourself. Applying
stomach, pancreas, spleen and
pressure to any small cut or
intestines.
wound with a sterile bandage will
stop the bleeding. Aspirin can • The cerebral vein – this drains
make the symptoms of asthma blood from the brain.
worse for those who suffer with it.
In addition, some people can be
allergic to aspirin.
Your haematologist will tell you if
aspirin is safe for your particular
situation and may suggest other
similar medications, such as
dipyridamole or clopidogrel, if
necessary.
Anticoagulation treatment
Long-term anticoagulation
treatment uses medication, also
referred to as blood-thinners,
to help prevent blood clots. This
can be started if you have had
Helpline freephone 08088 010 444 15Living with ET
After a diagnosis of ET, you may make changes to your lifestyle
find that it affects you both to try to stay as well as possible
physically and emotionally. This after your diagnosis and during
section will talk about both of treatment. Don’t try to change too
these aspects. much at once. Adopting a healthy
way of living is about making
Emotional impact of ET small, manageable changes to
Being told you have cancer can your lifestyle.
be very upsetting. Although the A healthy lifestyle includes having
outlook for many ET patients is a a well-balanced diet and being
positive one, it is a blood cancer physically active. With some
and a rare condition and, because of the side effects you may be
of this, you may need emotional, experiencing, the idea of getting
as well as practical support. Being out and being active may be the
diagnosed with a rare disease can last thing you want to do, but it
affect the whole of you, not just is important to try and stay as
your body, and can impact you active as possible to make you
emotionally at any point of your feel better and reduce some of the
journey. It is likely that you will symptoms or side effects you may
experience a range of complex be experiencing.
thoughts and emotions, some
of which may feel strange or
unfamiliar to you. It is important "People with an MPN
to know that these feelings are all should not smoke
valid and a normal response to and should exercise
your illness. to maintain their
general fitness and
It is important to remember that, improve their vitality
with current treatments, you can and cardiovascular
expect a good response and to live performance – this
a long, normal life. also reduces their
risk of cardiovascular
Looking after you disease." – Professor
You can live a long and normal Claire Harrison
life with ET, but you may want to
16 www.leukaemiacare.org.ukOne of the most commonly • Discuss your fatigue with your
reported side effects from the doctor or nurse.
treatment of ET is fatigue. This
isn’t normal tiredness and doesn’t Practical support
improve with sleep. Work and finances
Some general tips on how to deal Being diagnosed with ET can
with fatigue include: sometimes lead to difficulties
relating to your work life. Your
• Have a regular lifestyle – try diagnosis may lead to temporary
going to bed and waking up
sick leave or a reduction in
approximately the same time
working hours but it can also
every day and try to avoid lying
mean that you have to stop work
in.
altogether. You may need to
• Take part in regular, gentle make an arrangement with your
exercise to maintain your employer for times when you may
fitness levels as much as need to go into hospital or for
possible. those times when you may not be
well enough to go into work.
• Reserve your energy for what
you find important and build Your consultant or your GP can
rest periods around those times. arrange letters for your employer
to confirm your diagnosis and the
• Before going to bed, avoid effects it may have on your work
stimulants such as alcohol, life. It is often worth taking time to
coffee, tea or chocolate, or explain ET to your employer, as it
using laptops, tablets or mobile is likely they will never have heard
phones. of the disease before.
• Keep your bedroom quiet and at It is important for you to know
a comfortable temperature. that people with any form of
• Talk about your worries with cancer are covered by law by
the Equality Act. This means
family, friends, or your doctor
or nurse, or patient support that legally your employer
groups. cannot discriminate against
you and must make reasonable
Helpline freephone 08088 010 444 17Living with ET (cont.)
arrangements and adjustments
relating to your disease.
If you would like advice about
some of the financial help
available to you, then you can
speak to our Patient Advocacy
team on 08088 010 444.
Alternatively, Macmillan has
published a booklet about
financial support following a
diagnosis of cancer that might
be useful to you. They can also
give you personal advice over the
phone via their helpline at 0808
808 000 and you can discuss
which benefits you are eligible
for. Some Macmillan centres can
arrange face-to-face meetings
with a benefits advisor. They can
also provide financial assistance
in the form of grants – ask your
nurse in the hospital how to apply.
As ET is regarded as a cancer, you
will also be entitled to apply for
a medical exemption certificate
which means that you are entitled
to free NHS prescriptions in
England and Wales. Your GP or
specialist nurse at the hospital
can provide you with the details of
how to apply for this.
18 www.leukaemiacare.org.ukHelpline freephone 08088 010 444 19
Talking about ET
Talking to your • Be open and honest when
you discuss your symptoms
haematologist and how you are coping. Good
ET is a rare condition. It is patient-doctor communication
important for you to develop a tends to improve outcomes for
good working relationship with patients.
your haematologist so you are
given the best treatment possible • Bring someone along to your
for you. appointment. They can provide
support, ask questions and take
Here are some tips for working notes.
well with your haematologist:
• Don’t be afraid to ask for
• If it is an initial consultation, a second opinion – most
take along a list of your current haematologists are happy for
medications and doses, and you to ask.
a list of any allergies you may
have. You need to tell your
• If you have a complicated haematologist if...
medical history, take a list of You’re having any medical
diagnoses, previous procedures treatment or taking any products
and/or complications. such as prescribed medicines,
over the counter treatments
• Make a list of questions to take or vitamins. It is important to
to your appointment. This will understand that treatments,
help the discussion with your including complementary
haematologist. therapies, which are perfectly
• It can be useful to repeat back safe for most people, may
what you have heard so that you not be safe if you are being
can be sure that you have fully treated for ET. Remember, if
understood. you want to start any form of
complementary therapy outside
• Note down information to help of your medical treatment,
you remember what was said. consult your haematology
consultant or clinical nurse
20 www.leukaemiacare.org.ukspecialist prior to beginning it. terms of what others say and do.
It is important to understand Often people make assumptions
the difference between and do what they think helps. For
complementary therapies, used example, saying you look well,
alongside standard treatment, recounting stories of others they
and alternative therapies, used know with a similar diagnosis or
instead of standard treatment. encouraging you to look ahead
There is no evidence to suggest and stay positive, isn’t always
that any form of alternative what people really want to hear.
therapy can treat ET.
In many ways, the more you
Talking to other people communicate with them the
better. However, it is perfectly
Telling people you have a rare natural if you feel at some points
condition like ET can be hard to that you would like to keep some
explain. You might find it useful to things to yourself.
let your close family and friends,
as well as your employer, know These points may help you:
about your health condition. It
might be easier to provide people • Explain that you have a
condition that means your
with basic information and give
bone marrow does not function
them information leaflets about
properly, and this affects
ET if they want to know more in-
the number of blood cells it
depth details.
produces.
It is probably best to focus
conversations on the symptoms • Explain your symptoms (maybe
you are tired, or have a lot of
that you are experiencing, how
pain).
the condition affects you and
how you feel about it. Often • Explain what you need (maybe
people misunderstand and, more help day-to-day, or
unfortunately, it will mostly fall someone to talk to).
to you to educate them as best
as you can. Where possible, it’s
advisable to let people know what
you find helpful and unhelpful, in
Helpline freephone 08088 010 444 21Talking about ET (cont.)
You could also consider the who care will want to help you
following when telling people as best as they can. Talk as and
about your diagnosis: when you feel comfortable, so
those around you will know
• Find out more – Try to find when you need them most.
out as much as you can
about your condition from
reliable internet sources,
If you’re struggling to
charitable organisations or your
come to terms with your
consultant haematologist. The
diagnosis and prognosis,
more you know, the more you
you can speak to us on
can share.
our helpline. Call us on
08088 010 444.
• Have a print out to hand – It
may help to have a factsheet, or
a booklet like this one, to hand
to share with family and friends.
This will take the pressure
off you having to remember
everything they may want to
know.
• Explain your needs – Try and
be clear about what your needs
may be. Perhaps you need help
with the weekly food shop,
help with cooking dinner, or
someone to drive you to and
from appointments. You may
find that friends and family
are pleased that they can do
something to help you.
• Be open about how you feel –
Don’t be afraid of opening up
about how you feel, as people
22 www.leukaemiacare.org.ukGlossary
Acute Myeloid Leukaemia processes including regulation of
(AML) calcium, cell adhesion and gene
expression. Mutations in CALR are
A rapid and aggressive cancer
common in ET and MF.
of the myeloid cells in the bone
marrow. Chromosomes
Anaemia Thread-like structures which carry
the genes, and are located in the
A condition where the number of
nuclei of every cell in the body.
red blood cells are reduced. Red
There are 46 chromosomes (23
blood cells contain haemoglobin
pairs) in humans.
and transport oxygen to body
cells. This may be due to a lack Chronic Myeloid Leukaemia
of iron, leukaemia, or sickle cell (CML)
disease.
A leukaemia in which the myeloid
Anticoagulation cells start multiplying in the bone
marrow leading to large numbers
The process of administering
of abnormal, immature myeloid
anticoagulants which are drugs
cells called blasts, which prevent
to prevent the blood from clotting
the bone marrow from producing
unnecessarily.
enough healthy blood cells of all
Bone Marrow types.
A soft blood-forming tissue that First-line Treatment
fills the cavities of bones and
The first treatment given for
contains fat, immature and
a disease. It is generally the
mature blood cells, including
treatment accepted by the
white blood cells, red blood cells
medical establishment for initial
and platelets.
treatment of a given type and
Calreticulin (CALR) stage of cancer.
A soluble protein which is known
to be involved in many body cell
Helpline freephone 08088 010 444 23Glossary (cont.)
Genes Myelofibrosis (MF)
Genes are made up of DNA which A reactive and reversible
stores the genetic information process which occurs with many
required to make human proteins. cancerous and non-cancerous
diseases of the bone marrow.
Irradiation
Particles or rays falling on to a Platelets
surface (radiation wave on the One of the types of blood cells
surface of the skin). which help to stop bleeding.
Leukaemia Polycythaemia Vera (PV)
A group of cancers that usually A chronic condition belonging
begin in the bone marrow to the myeloproliferative
and result in high numbers of neoplasms group of diseases. It
abnormal blood cells. These cells is characterised by too many red
are not fully developed and are blood cells, and sometimes too
called blasts or leukaemia cells. many platelets and white cells, in
Depending on the type of blood the blood.
cell involved, there are different
types of leukaemia with varying Prognosis
characteristics, such as being An indication of how well a
acute (develops quickly) or patient is expected to respond
chronic (develops slowly). to treatment based on their
individual characteristics at
Myeloproliferative Neoplasms the time of diagnosis or other
(MPNs) timepoint in the disease.
A disease of the bone marrow in
which excess cells are produced.
Pulmonary Embolism
A blockage of a blood vessel in the
Myeloid lung. It can be secondary to a clot
Relates to bone marrow. elsewhere in the body which then
travels up to the lung.
24 www.leukaemiacare.org.ukRed Blood Cells excessive number of platelets in
the blood (a platelet count greater
Small blood cells that contain
than 450x109/L). Platelets are
haemoglobin and carry oxygen
blood cells in plasma that stop
and other substances to all
bleeding by sticking together to
tissues of the body.
form a clot. Too many platelets
Second-line Treatment can lead to certain conditions,
including stroke, heart attack, or a
Treatment other than the type
clot in the blood vessels.
used the first time (first-line
treatment). Thrombosis
Spleen Clotting or coagulation of the
blood in a part of the circulation
The largest organ of the lymphatic
in both arteries and veins.
system whose function is to help
rid the body of toxins, waste and von Willebrand syndrome
other unwanted materials. The
A common hereditary blood-
spleen is located under the ribs
clotting disorder which causes
on the left of the abdomen.
varying degrees of bleeding. It
Stem Cell can be hereditary or acquired.
In acquired von Willebrand
The most basic cell in the body
syndrome, the patient has auto
that has the ability to develop
antibodies to the von Willebrand
into any of the body’s specialised
clotting factor and this often
cell types, from muscle cells to
occurs in conjunction with
brain cells. However, what makes
another autoimmune disease
these stem cells reproduce
such as rheumatoid arthritis.
uncontrollably, as in cancer,
is thought to be linked to White blood cells
chromosome abnormalities.
White blood cells are one of the
Thrombocytosis types of cells found in the blood
and bone marrow, along with red
A condition in which there is an
Helpline freephone 08088 010 444 25Glossary (cont.)
blood cells and platelets. White
blood cells create an immune
response against both infectious
disease and foreign invaders.
Granulocyte white blood cells,
include the neutrophils (protect
against bacterial infections
and inflammation), eosinophils
(protect against parasites and
allergens) and basophils (create
the inflammatory reactions
during an immune response).
Other white blood cells include
the lymphocytes (recognise
bacteria, viruses and toxins, to
which they produce antibodies)
and monocytes (clear infection
products from the body).
26 www.leukaemiacare.org.ukUseful contacts
and further support
There are a number of helpful Blood Cancer UK
sources to support you during Blood Cancer UK is the leading
your diagnosis, treatment and charity into the research of blood
beyond, including: cancers. They offer support to
• Your haematologist and patients, their family and friends
healthcare team through patient services.
• Your family and friends 0808 2080 888
• Your psychologist (ask your www.bloodcancer.org.uk
haematologist or CNS for a
referral)
Cancer Research UK
Cancer Research UK is a leading
• Reliable online sources, charity dedicated to cancer
such as Leukaemia Care
research.
• Charitable organisations 0808 800 4040
There are a number of www.cancerresearchuk.org
organisations, including
ourselves, who provide expert Macmillan
advice and information. Macmillan provides free practical,
medical and financial support for
Leukaemia Care
people facing cancer.
We are a charity dedicated to
0808 808 0000
supporting anyone affected by
www.macmillan.org.uk
the diagnosis of any blood cancer.
We provide emotional support Maggie’s Centres
through a range of support Maggie’s offers free practical,
services including a helpline, emotional and social support
patient and carer conferences, to people with cancer and their
support group, informative families and friends.
website, one-to-one buddy
service and high-quality patient 0300 123 1801
information. We also have a nurse www.maggiescentres.org
on our help line for any medical Citizens Advice Bureau (CAB)
queries relating to your diagnosis.
Offers advice on benefits and
Helpline: 08088 010 444 financial assistance.
www.leukaemiacare.org.uk
support@leukaemiacare.org.uk 08444 111 444
www.adviceguide.org.uk
Helpline freephone 08088 010 444 27Leukaemia Care is a national charity dedicated to providing information, advice and support to anyone affected by a blood cancer. Around 34,000 new cases of blood cancer are diagnosed in the UK each year. We are here to support you, whether you’re a patient, carer or family member. Want to talk? Helpline: 08088 010 444 (free from landlines and all major mobile networks) Office Line: 01905 755977 www.leukaemiacare.org.uk support@leukaemiacare.org.uk Leukaemia Care, One Birch Court, Blackpole East, Worcester, WR3 8SG Leukaemia Care is registered as a charity in England and Wales (no.1183890) and Scotland (no. SCO49802). Company number: 11911752 (England and Wales). Registered office address: One Birch Court, Blackpole East, Worcester, WR3 8SG
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