Acute Promyelocytic Leukaemia (APL) - A Guide for Patients
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Acute Promyelocytic Leukaemia (APL) A Guide for Patients
Introduction
Being diagnosed with acute promyelocytic leukaemia
(APL) can be a shock, particularly when you may never
have heard of it. If you have questions about APL – 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
about how to get the best from your haematologist, plus
practical advice on how to help important people in your
life understand such a rare condition.
For more personalised peer reviewed by Nigel Russell,
information, talk to your Professor of Haematology at
haematologist, clinical nurse University of Nottingham, and
specialist or hospital pharmacist. James Allan, Professor of Cancer
Genetics at Newcastle University.
This booklet was originally
We are also grateful to Thea
compiled by Ken Campbell, MSC
Wilson for her contributions as a
(Clinical Oncology) and peer
patient reviewer.
reviewed by Dr George Cherian
and Professor David Grimwade, Throughout this booklet, you will
who provided additional support. see a number of quotations. These
The rewrite was put together by are the real experiences of blood
Lisa Lovelidge and updated by cancer patients and may help you
our Patient Information Writer, to understand your disease and
Isabelle Leach. It has been situation a bit better.
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 2
Printed: 01/2020
2 www.leukaemiacare.org.uk Review date: 01/2022In this booklet
Introduction 2
In this booklet 3
About Leukaemia Care 4
What is APL? 6
Symptoms and diagnosis of APL 8
Treating with APL 10
Living with APL 18
Talking about APL 22
Glossary 25
Useful contacts and further support 27
Helpline freephone 08088 010 444 3About Leukaemia Care
Leukaemia Care is a national charity dedicated to ensuring
that people affected by blood cancer have access to the
right information, advice and support.
Our services has been affected by a blood
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Helpline freephone 08088 010 444 5What is APL?
Acute promyelocytic leukaemia to all tissues of the body.
(APL) is a rare sub-type of acute
myeloid leukaemia (AML) in which
2. Platelets that form blood clots
to stop bleeding.
there is an increased production
of immature, abnormal white 3. White blood cells that fight
blood cells called promyelocytes infection and disease; the
in the bone marrow. The term shortest-lived white cells are
acute indicates that the called neutrophils.
leukaemia develops rapidly rather
than being a description of its A lymphoid stem cell becomes a
seriousness. lymphoblast cell and then one of
three types of lymphocytes (white
To understand APL, it is helpful blood cells):
to understand how blood cells
are normally produced. Blood 1. B-lymphocytes that make
cells are produced in the bone antibodies to help fight
marrow, which is spongy tissue infection.
found inside bones. Each day, 2. T-lymphocytes that help
the bone marrow produces more B-lymphocytes make the
than a trillion new blood cells to antibodies that help fight
replace those that are worn out. infection.
Blood stem cells divide to produce
either mature blood cells or more 3. Natural killer cells that attack
stem cells. Only about one in 5000 cancer cells and viruses.
of the cells in the bone marrow is
In APL, the myeloid cells that are
a stem cell. A blood stem cell, also
destined to mature into white
called a haematopoietic stem cell,
blood cells (through a process
may become a myeloid stem cell
called differentiation) fail to
or a lymphoid stem cell.
do so and remain as immature
A myeloid stem cell becomes one promyelocyte cells.
of three types of mature blood
These promyelocyte cells
cell:
accumulate in the bone marrow
1. Red blood cells that carry leading to a shortage of normal
oxygen and other substances white and red blood cells and
6 www.leukaemiacare.org.ukplatelets which cause the gene on chromosome 17 swap and
symptoms of APL. fuse to become the PML-RARA
gene.
How common is APL?
A small number of APL patients
APL is considered a rare disease (less than 10%) do not have the
as it affects about two people PML-RARA gene but have other
in every million per year. It rarer gene fusions. APL can also
represents approximately 10% of develop after receiving certain
all cases of AML. APL affects men types of chemotherapy or
and women equally. There are radiotherapy given for another
about 160 new cases diagnosed in cancer. This type of APL is called
the UK each year. treatment-related APL. The risk of
APL can be diagnosed at any age. developing APL is highest around
The median age at diagnosis is 51 three years after the treatment for
years. the prior cancer.
What causes APL?
The exact cause of APL is not
known. However, APL is commonly
associated with the PML-RARA
gene which is sometimes called
the hallmark of APL. The PML-
RARA gene is created during
a person’s lifetime and is not
passed on to children. It is the
result of a translocation between
chromosome 15 and chromosome
17. A translocation is the transfer
of one part of a chromosome to
another part of the same or a
different chromosome, resulting
in rearrangement of the genes.
In the case of APL, the PML gene
on chromosome 15 and the RARA
Helpline freephone 08088 010 444 7Symptoms and diagnosis of APL
Symptoms of APL DIC may happen in other forms of
leukaemia but is rare. It affects
The most common symptoms about four out of five patients
of APL are similar to those seen with APL. DIC happens when the
in other acute leukaemias and body forms clots and breaks down
are caused by the bone marrow clots inside the blood vessels. This
failing to produce enough normal uses up the clotting factors in the
blood cells. Most patients will blood and this can lead to severe,
experience shortness of breath even life-threatening, bleeding.
and tiredness with slight exercise, Fortunately, modern treatments
which is caused by a shortage are very effective in blocking DIC.
of red blood cells (anaemia).
Anaemia means the body cannot To summarise, common
supply enough oxygen to muscles symptoms and their causes are:
and other tissues. Infections are
common and often persistent
• Anaemia – breathlessness,
easily tired
because of a deficiency in
neutrophils, a white blood cell • Low white blood cells –
that normally helps to fight frequent and persistent
infection. infections
Bruising and bleeding are often • Low platelets – bruising and/or
seen, and can range from slight bleeding
bruises in the skin to serious
internal bleeding. This is caused • DIC – bruising/bleeding which
partly by a shortage of platelets, may be very severe
but also by a condition called
disseminated intravascular
Diagnosis of APL
coagulation (DIC). Platelets help If APL is suspected, you’ll have
the body to form a clot at the site a set of tests to confirm the
of bleeding, so when there are too diagnosis. If you’re diagnosed
few, this may cause bruising or with APL, you will have further
slight bleeding. tests to determine the right
treatment for your cancer. It’s
important that you know and
8 www.leukaemiacare.org.ukunderstand your diagnosis, so you and RARA are two genes which
can ask questions and be fully are normally found on different
informed of what to expect. chromosomes. In APL, the two
chromosomes swap over part of
Full blood count their DNA, which joins the PML
A blood sample is examined and RARA genes together. This
using an automatic cell-counting test is important because the
machine and by examining a main drugs used to treat APL work
stained film under a microscope. directly on the PML-RARA gene. In
The cell-counter will usually the very rare cases of APL without
indicate that there are large the gene, other treatments can be
numbers of abnormal white cells used.
in the blood. The appearance of
There are also a number of other
the stained blood cells is usually
special investigations that can
very typical of APL.
be done to confirm the diagnosis
Bone marrow examination of APL and to help in planning
If the results of a blood sample treatment. Unlike most forms of
show that you may have APL, or leukaemia, treatment of APL often
another form of leukaemia, a bone starts before all the tests are
marrow sample will be taken, completed.
usually from the hip bone. This is These tests may be repeated
done under a local anaesthetic from time to time during your
and does not take very long. The treatment. This is to find out
bone marrow sample is important how the APL is responding to
to confirm the diagnosis and also treatment.
for comparison with later samples
to show how APL is responding to "Lots of things were thrown at me
treatment. in a short amount of time but the
sentence I clung on to was ‘we
Additional investigations are waiting confirmation, but the
There is a test that looks for an leukaemia we think you have carries
abnormality called PML-RARA. This a high cure rate. We should know by
is an abnormal fusion gene – PML the morning.’ I remember thinking,
please, please let it be that one."
Helpline freephone 08088 010 444 9Treating APL
APL is treated in a very different symptoms. A firm diagnosis of
way from other forms of AML; APL using genetic testing can be
if a patient with APL is given performed later, and treatment
standard treatment, there is a can be discontinued if APL is not
risk of serious problems with confirmed.
their clotting system. Fortunately,
Patients with APL are generally
it is usually very easy to tell the
subdivided into the following
difference between APL and other
two groups according to their
types of AML.
white blood count as treatment
APL usually responds very well to recommendations can differ for
treatment and patients with this each group:
form of leukaemia have a good
chance of being cured.
1. Low- to intermediate-risk:
patients with a white blood
APL can affect people of any cell count of 10,000 cells per
age, but this booklet is about microlitre of blood or less.
APL in adults. If you are a parent
of a child with APL, you should
2. High-risk: patients with a
white blood cell count of
ask their specialist about the
more than 10,000 cells per
differences in treatment and
microlitre of blood.
outlook for children.
If you have any concerns, contact First-line treatment
your haematologist. First-line treatment for APL
includes all-trans retinoic acid
Treatment options (ATRA), which is an active by-
Treatment of patients who are product of vitamin A. ATRA blocks
suspected of having APL should the effect of the PML-RARA gene
be treated immediately, even that prevents the promyelocyte
before the diagnosis is made, cells maturing into normal white
because they can quickly develop blood cells (differentiation). ATRA
potentially life-threatening is not a chemotherapeutic drug
bleeding or blood clotting and is called a differentiating
10 www.leukaemiacare.org.ukagent. It is given in combination it to be at least as effective as
with another drug in patients the combination of ATRA and
with APL to prevent any drug anthracyclines, if not more so,
resistance. with a reduced risk of disease
relapse.
ATRA can sometimes be given
with chemotherapy drugs called Induction treatment
anthracyclines. Anthracyclines,
To achieve remission (induction
such as daunorubicin and
therapy), the 2019 guidelines
idarubicin, interfere with the DNA
from the European Leukaemia
and reproduction of white blood
Network (European LeukemiaNet)
cells, including the leukaemia
recommend the following
cells. ATRA is given as a capsule,
regimens:
while anthracyclines are given
intravenously. Low-to-intermediate risk
patients: ATRA and ATO
In 2018, NICE approved a drug
called arsenic trioxide (ATO) for High-risk patients: Both of the
the first-line treatment of APL in following regimens achieve
previously untreated patients, similar results; however, ATO
with low- to intermediate-risk is not approved for high-risk
disease and patients whose APL patients by NICE as yet.
has returned (relapsed) or did
not respond to chemotherapy • ATRA and ATO plus a
cytoreductive chemotherapy
(refractory). ATO is also a
such as cytarabine.
differentiating agent and acts in a
Cytoreductive means that the
similar way to ATRA.
chemotherapy reduces the
Because differentiating agents number of cells.
have less side effects to
chemotherapy drugs, especially • ATRA plus anthracyclines. The
most frequently used regimen
anthracyclines, the combination
being called AIDA.
of ATRA and ATO alone is a
preferred first-line therapy, The treatment for APL that has
particularly as studies found developed as a consequence of
Helpline freephone 08088 010 444 11Treating APL (cont.)
prior chemotherapy is normally to three courses of anthracycline-
similar to APL associated with based chemotherapy should be
the PML-RARA gene, although given for consolidation therapy.
your doctor may choose to use a This is usually given as an
different drug in this situation. inpatient.
In addition to induction Minimal residual disease and
treatment, patients with APL molecular monitoring
require supportive care in
After consolidation treatment
the form of blood product
has been completed, a bone
transfusions to maintain the
marrow molecular assessment
platelet count and the blood
is recommended to confirm
clotting indicators as normal
complete response (CR) with
as possible and to prevent the
negative minimal residual
risk of bleeding. Blood chemical
disease (MRD):
levels (particularly potassium and
magnesium which are important • Complete response (CR),
for electrical conduction in the also known as complete
heart) will be monitored closely. remission – This is based
Sometimes it is necessary to on morphological remission,
also give potassium and/or meaning that a patient has a
magnesium supplements. normal blood count, a normal
development of blood cells in
Consolidation treatment the bone marrow and their blast
To consolidate remission count is at less than 5%.
in patients who have not
received chemotherapy-based • Minimal residual disease
treatment, four courses of ATO (MRD) - The presence of small,
and seven courses of ATRA are but measurable, numbers of
recommended. This can usually leukaemia cells in the blood
be given as an outpatient. of a patient who does not have
any clinical presentation of a
For patients who received ATRA blood cancer (i.e. the patient
and chemotherapy regimens, two is in remission). If there are
12 www.leukaemiacare.org.ukno detectable leukaemia cells successful in most patients with
found, then a patient is said to APL. However, for patients who
be MRD-negative. haven’t gone into first remission
or who have relapsed, second-line
This is then to be followed by
treatment options are available.
routine monitoring for certain
patients, depending on their CR Relapse or being refractory to
and MRD results as well as their first-line treatment can occur in
risk classification: any patient with APL, regardless
of whether they have been treated
• Routine monitoring may not be with ATRA with ATO or ATRA with
necessary for low-risk patients
chemotherapy. However, these
with CR with negative MRD after
events are uncommon in low- to
consolidation.
intermediate-risk patients.
• MRD monitoring every three The second-line treatment you
months for up to three
have for relapsed or refractory APL
years after consolidation is
will depend mainly on which first-
recommended for high-risk
line treatment you were given. If
patients.
you have had ATRA with ATO as
Maintenance treatment first-line treatment, then you will
receive ATRA with chemotherapy,
For low- to intermediate-risk
and vice-versa (you will be given
patients, maintenance treatment
ATRA with ATO if you had ATRA
after consolidation with ATO and
with chemotherapy as first-line
ATRA is not recommended, but for
treatment).
high-risk patients on ATRA and
chemotherapy who are showing In young, fit patients, an
clinical benefit, maintenance may autologous stem cell transplant
be initiated with tablets for two can be performed. With an
years. autologous stem cell transplant,
you are given intensive
Second-line treatment chemotherapy to destroy all the
First-line treatment is generally leukaemia cells. However, as the
Helpline freephone 08088 010 444 13Treating APL (cont.)
chemotherapy will also kill your blood cells, red blood cells and
own bone marrow cells, you are platelets)
given a transplant of your own
healthy stem cells which were Differentiation syndrome
collected before the intensive Previously known as retinoic
chemotherapy. However, in acid syndrome (or RA syndrome),
patients who were responding differentiation syndrome can
well to ATO and then relapsed, affect some patients being
a transplant is not always treated with ATRA, ATO or both. It
necessary. occurs when these differentiating
agents start allowing the
Side effects promyelocyte cells to mature.
Side effects in patients with The exact mechanism involved is
APL when treated with ATRA unknown but it is thought to be
and ATO include differentiation an inflammatory response.
syndrome, pseudotumour cerebri, Differentiation syndrome is most
hyperleukocytosis, heart rhythm likely to happen during the first
disturbance (prolongation of QTc three weeks of treatment and is
interval) and signs of toxicity in more common in patients with a
the liver. high white blood cell count.
Side effects common Symptoms include:
with anthracyclines are
cardiomyopathy (disease of the • Fever
heart muscle) and secondary
leukaemia. • Cough
Other side effects include: • Breathing difficulty
• Nausea and vomiting • Fluid in the lungs
• Alopecia • Weight gain
• Bone marrow suppression • Fluid in the tissues
(resulting in low levels of white • Kidney damage
14 www.leukaemiacare.org.ukDifferentiation syndrome can Hyperleukocytosis,
usually be treated with steroids. leukostasis and disseminated
Treatment with differentiating intravascular coagulation
agents may be temporarily
Hyperleukocytosis is said to
reduced or suspended, but
occur when the white blood cell
this would only happen if the
count is greater than 100,000 per
differentiation syndrome is very
microlitre. This highly increased
severe, which is rarely the case.
white blood cell count often
Most current ATRA with ATO
leads to complications such as
regimens include preventative
leukostasis and disseminated
steroid treatment.
intravascular coagulation.
Pseudotumour cerebri
Leukostasis occurs when the
Pseudotumour cerebri is seen excessive numbers of white blood
with both ATRA and ATO, and cells make the blood viscous and
is more common when these prone to clotting.
two differentiating agents are
given together. Pseudotumour Disseminated intravascular
cerebri is a non-harmful increase coagulation (DIC) is a condition
in pressure in the skull and where the small blood clots
unrelated to any tumour. develop throughout the
bloodstream and block blood
Symptoms include: vessels. Because the increased
clotting uses up all the platelet
• Headaches cells and clotting factors to
• Swelling of the optic disk control bleeding, patients are
prone to excessive bleeding. This
• Double vision is a very serious complication
• Confusion which should be treated as soon
as possible.
If it occurs, pseudotumour cerebri
can be treated with steroids and In order to prevent DIC from
by decreasing the dose of ATRA. developing, patients with
hyperleukocytosis should be
Helpline freephone 08088 010 444 15Treating APL (cont.)
treated with anthracycline- What happens if these
based chemotherapy. The use
of the chemotherapy drug
drugs do not work for
hydroxycarbamide should be me?
considered for treatment of For the majority of patients
leukocytosis (a white blood cell who do not respond to first-line
count greater than 100,000 per treatment with ATRA and ATO, or
microlitre) in patients treated ATRA with ATO and cytarabine in
with combination therapy of ATRA high-risk patients, or patients
and ATO to keep the white blood who relapse at a later point, a
cell count low. good response to second-line
treatment is generally achieved
Heart rhythm disturbances
with ATRA and anthracyclines,
Some patients treated with ATRA, or ATRA with ATO if they received
ATO or both might show heart ATRA with anthracyclines as
rhythm disturbances such as a first-line treatment. While
prolongation of the QTc interval relapse or being refractory to
on their electrocardiogram first-line treatment can occur
(ECG) which may cause heart in any patient with APL, this is
problems, especially for patients an uncommon event in low- to
with existing heart disease. intermediate-risk patients.
Therefore, ECG monitoring before
and twice a week throughout In the event of requiring second-
treatment is recommended. For line treatment, it is worth
patients who show prolongation considering an autologous stem
of the QTc interval on their ECG, cell transplant in patients who
treatment should be stopped and are fit enough to withstand the
restarted when the heart rhythm procedure.
disturbances subside. Your consultant will be able to
discuss these treatments with
you, and any circumstances
in which they might become
necessary.
16 www.leukaemiacare.org.ukPrognosis of APL
For patients who are diagnosed
and start treatment promptly,
the outcome of APL is very good.
Since the introduction of the
combination of ATRA and ATO, cure
rates for APL of approximately
90% for low- to intermediate-risk
and 85% for high-risk patients
have been reported.
An important remaining issue
is early death which occurs in
approximately 10-15% of patients
who do not receive immediate
treatment. Efforts in this area are
focusing on education to improve
prompt diagnosis and early
management of patients with
suspected APL.
Patients who are older, male,
have problems with their kidneys
(measured by serum creatinine
levels) and increased levels of
fibrinogen (protein involved in
clotting the blood) have a worse
prognosis.
Helpline freephone 08088 010 444 17Living with APL
Being diagnosed with an aggressive form of leukaemia like APL
can be difficult, both practically and emotionally. This chapter
will talk about both of these aspects.
Emotional impact of know your limits and not try to
change too much at once. Exactly
APL what you can do will vary and
Being told you have cancer can be will depend on the treatment you
very upsetting. It can be especially have had, and how fit you were
difficult with acute leukaemia before your leukaemia. Adopting
as you often get ill suddenly and a healthy way of living is about
have to start treatment quickly. making small, manageable
There is usually very little time to changes to your lifestyle.
take in information and start to
cope with it. You can find more information
about living well with
APL is a rare condition and, leukaemia on our website: www.
because of this, you may need leukaemiacare.org.uk/support-
emotional, as well as practical, and-information/information-
support. Being diagnosed with about-blood-cancer/living-well-
a rare disease can impact you with-leukaemia/
emotionally at any point of your
journey. It is likely that you will Diet
experience a range of complex Diet plays an important part
thoughts and emotions, some in coping with cancer and its
of which may feel strange or treatment and recovery. A well-
unfamiliar to you. It is important balanced diet can help you feel
to know that these feelings are all stronger, have more energy, and
valid and a normal response to recover quicker.
your illness.
If you’re having treatment,
Looking after you you may notice that you lose
weight, or your taste or appetite
Following a diagnosis of APL, you
changes. This may be due to the
may wish to make changes to
side effects of your treatment
your lifestyle. It’s important to
18 www.leukaemiacare.org.ukincluding a sore mouth or nausea ordinary infections may occur
and sickness. more often and be more severe or
longer lasting. You may also get ill
Once your treatment has finished
from infections with germs that
though, you should begin to feel
normally live in your body without
better and be able to eat a normal
causing problems, but which grow
diet. This can take a while after
more rapidly when your immune
intensive treatment.
system is not working – these are
Exercise called opportunistic infections.
With some of the side effects The neutropenic diet can help
you may be experiencing, such protect patients with weakened
as fatigue, the idea of getting immune systems.
out and being active may be the
If you think you may have an
last thing you want to do, but it
infection, you should contact your
is important to try and stay as
doctor straightaway. Common
active as possible. This will help to
symptoms of infection include:
make you feel better and reduce
some of the symptoms or side • Fever – a raised temperature
effects you may be experiencing. (38°C or higher)
Speak to your clinical nurse
specialist about exercises that • Aching muscles
may be suitable for you.
• Diarrhoea
Infection • Headaches
One of the most common
problems following a diagnosis • Excessive tiredness
of APL is infection. When you have The signs and symptoms of
APL, your body is not able to fight infection may be less obvious
infections as well as normal – this when you have APL, so if you are
is known as immunosuppression. in any doubt it is best to contact
If you have immunosuppression, your doctor and ask for advice.
Helpline freephone 08088 010 444 19Living with APL (cont.)
You can help to reduce the risk of is recommended by someone
infection by taking some simple other than your APL specialist, you
precautions: should check that it is safe first.
• Wash your hands frequently, Shingles
especially after using the
If you have previously been
toilet, and also if you have
exposed to chickenpox, you may
touched something like a door
develop a painful nerve condition
knob or banister which can
called shingles. Even if it was
be contaminated with lots of
a long time ago, the virus can
germs.
live dormant for many years
• Try not to spend unnecessary and surface when your immune
time in crowds; especially if system is supressed. You may
there is an epidemic of flu or be able to receive a vaccine
another illness. against shingles but, as it is a live
vaccine, you should talk to your
• You should be very careful to doctor about this.
follow food safety advice, such
as cleanliness in the kitchen Practical support
and not keeping food after use-
Work and finances
by dates.
Being diagnosed with APL means
Vaccines you will need to start treatment
Vaccinations may not work as well straightaway and so you, or
when you have leukaemia, but it is someone you know, will need to
still recommended that you have contact your employer to inform
your annual flu vaccine. This will them of your situation. Your
still reduce the risk of getting ill condition will mean that you will
and will offer you some protection. need to be at hospital frequently
at first and you will need to make
APL patients should avoid having the appropriate arrangements
live vaccines which are used for with your employer with regard to
measles, mumps and rubella your working arrangements.
(MMR) and shingles. If a vaccine
20 www.leukaemiacare.org.ukYou may need to negotiate a help available to you, then
reduction in working hours or you can speak to our Patient
make an arrangement with your Advocacy team on 08088 010
employer for times when you have 444. Alternatively, Macmillan
to go into hospital or for those has published a booklet about
times when you may not be well financial support following a
enough to go into work. diagnosis of cancer that might
be useful to you. They can also
Your consultant or your GP can
give you personal advice over the
arrange letters for your employer
phone via their helpline at 0808
to confirm your diagnosis and the
808 0000 and you can discuss
effects it may have on your work
which benefits you are eligible
life. It is often worth taking time to
for. Some Macmillan centres can
explain APL to your employer, as it
arrange face-to-face meetings
is likely they will have never heard
with a benefits advisor. They can
of the disease.
also provide financial assistance
You could provide them with in the form of grants – ask your
a copy of this booklet or invite nurse in the hospital how to apply.
them to download it from the
As APL is a type of cancer, you
Leukaemia Care website at www.
will also be entitled to apply for
leukaemiacare.org.uk
a medical exemption certificate,
It is important for you to know which means that you are entitled
that people with any form of to free NHS prescriptions. Your GP
cancer are covered by law by or clinical nurse specialist at the
the Equality Act. This means hospital can provide you with the
that legally your employer details of how to apply for this. If
cannot discriminate against you are undergoing chemotherapy
you and must make reasonable you may qualify for a Blue Badge
arrangements and adjustments to help with hospital car parking.
relating to your disease. To apply for a badge, contact your
local council.
If you would like general advice
about some of the financial
Helpline freephone 08088 010 444 21Talking about APL
Talking to your • Note information down to help
you remember what was said.
haematologist
APL is a rare condition. It is • Be open when you discuss your
important for you to develop a symptoms and how you are
good working relationship with coping. Good patient-doctor
your haematologist, so you are communication tends to
given the best treatment possible. improve outcomes for patients.
The following gives advice Other tips:
on working well with your • Bring someone along to your
haematologist: appointment. They can provide
• If it’s an initial consultation, support, ask questions and take
notes.
take along a list of your current
medications and doses, and • Don’t be afraid to ask for
a list of any allergies you may a second opinion – most
have. haematologists are happy for
• If you have a complicated you to ask.
medical history, take a list of
diagnoses, previous procedures
You need to tell your
and/or complications. haematologist if...
You’re having any medical
• Make a list of questions to take treatment or taking any products
to your appointment. This will
such as prescribed medicines,
help the discussion with your
over the counter treatments
haematologist.
or vitamins. It is important to
• It can be useful to repeat back understand that treatments,
what you have heard so that including complementary
you can be sure that you fully therapies, which are perfectly safe
understood. for most people, may not be safe if
you are being treated for APL.
22 www.leukaemiacare.org.ukRemember, if you choose to start Talking to other people
any form of complementary
therapy outside of your medical Telling people you have a rare
treatment, consult your condition like APL can be hard.
haematology consultant or However, you might find it useful
clinical nurse specialist prior to let your close family and
to taking it. It is important friends, as well as your employer,
to understand the difference know about your health condition.
between complementary It might be easier to provide
therapies, used alongside people with basic information and
standard treatment, and give them information leaflets or
alternative therapies, used booklets like this one about APL if
instead of standard treatment. they want to know more in-depth
There is no evidence that any form details.
of alternative therapy can treat "I made a conscious decision to be
APL. very open about my illness. Telling
For help with talking to your family was tough. But I encouraged
haematologist, you can access people to ask questions."
more information about APL, It is probably best to focus
including a section on ‘Questions conversations on the symptoms
to ask your medical team’ at www. that you are experiencing, how
leukaemiacare.org.uk/support- the condition affects you and
and-information/information- how you feel about it. Often
about-blood-cancer/blood- people misunderstand and,
cancer-information/leukaemia/ unfortunately, it will mostly fall
acute-promyelocytic-leukaemia to you to educate them as best
which features a list of questions as you can. Where possible, it’s
that you may want to ask. advisable to let people know what
you find helpful and unhelpful, in
terms of what others say and do.
Often people make assumptions
Helpline freephone 08088 010 444 23Talking about APL (cont.)
and do what they think helps. For reliable internet sources,
example, saying you look well, charitable organisations or your
recounting stories of others they consultant haematologist. The
know with a similar diagnosis, more you know, the more you
and encouraging you to look can share.
ahead and stay positive isn’t
always what you really want to • Have a print out to hand
– It may help to have some
hear. In many ways, the more
information to hand to share
you communicate with them the
with family and friends. This will
better.
take the pressure off you having
These points may help you: to remember everything they
may want to know.
• Explain that you have a
condition that means your • Explain your needs – Try and
bone marrow does not function be clear about what your needs
properly, and this affects may be. Perhaps you need help
the number of blood cells it with the weekly food shop,
produces. help with cooking dinner, or
someone to drive you to and
• Explain your symptoms (maybe from appointments. You may
you are tired, or have a lot of
find that friends and family
pain).
are pleased that they can do
• Explain what you need (maybe something to help you.
more help day-to-day, or
someone to talk to).
• Be open about how you feel –
Don’t be afraid of opening up
You could also consider the about how you feel, as people
following when telling people who care will want to help you
about your diagnosis: as best they can. Talk as and
when you feel comfortable, so
• Find out more – Try to find those around you will know
out as much as you can when you need them most.
about your condition, from
24 www.leukaemiacare.org.ukGlossary
Acute Myeloid Leukaemia (AML) Chromosome
A rapid and aggressive cancer X-shaped, thread-like structures
of the myeloid cells in the bone which carry the genes, and are
marrow. located in the nuclei of every
cell in the body. There are 46
Anaemia chromosomes (23 pairs) in
A condition where the number of human cells.
red blood cells are reduced. Red
blood cells contain haemoglobin Consolidation
and transport oxygen to body A treatment phase given to
cells. This may be due to a lack remove any leukaemia cells that
of iron, leukaemia or sickle cell are still present after induction
disease. treatment, as this will help
reduce the risk of the leukaemia
Autologous Stem Cell returning.
Transplant
A transplant of stem cells derived
Fatigue
from part of the same individual Tiredness and weakness
receiving them. rendering the patient unable to
work or perform usual activities.
Bone Marrow
A soft blood-forming tissue that
Genes
fills the cavities of bones and Genes are made up of DNA which
contains fat, immature and stores the genetic information
mature blood cells, including required to make human proteins.
white blood cells, red blood cells
and platelets.
Induction
The treatment phase intended to
Chemotherapy kill the majority of the leukaemia
Drugs that work in different ways cells in the blood and bone
to stop the growth of cancer cells, marrow, and to restore normal
either by killing the cells or by blood cell production.
stopping them from dividing.
Lymphoid Cell
A cell originating in the bone
Helpline freephone 08088 010 444 25Glossary (cont.)
marrow which will eventually Relapse Condition
become lymphocytes and
A relapse occurs when a patient
antibodies.
initially responds to treatment,
Maintenance but after six months or more,
the response stops. This is also
An ongoing treatment phase
sometimes called a recurrence.
intended to prevent any
leukaemia cells from recurring. Remission
Lower doses of therapy are given
Remission is said to have
often for years.
occurred when the blood cell
Neutropenia counts have returned to normal
and there are less than 5% of
Low numbers of neutrophils,
abnormal, immature leukaemia
making you more vulnerable to
cells still present in the bone
infections.
marrow. Complete remission is
Neutrophils said to have occurred when there
are no leukaemia cells anywhere
White blood cells involved in
else in the body.
fighting inflammation and
infection, specifically bacterial Stem Cells
infections.
The most basic cell in the body
Platelets that has the ability to develop into
any of the body’s specialised cell
Small blood cells that help the
types, from muscle cells to brain
body form clots to stop bleeding.
cells.
Red Blood Cells White Blood Cells (or
Small blood cells that contain Leukocytes)
haemoglobin and carry oxygen
The main role of white blood cells
and other substances to all
is creating an immune response
tissues of the body.
against both infectious disease
Refractory Condition and foreign invaders. There are
several different types of white
A condition for which treatment
blood cells, and each has a
does not result in a remission.
different role.
26 www.leukaemiacare.org.ukUseful contacts
and further support
There are a number of helpful Bloodwise
sources to support you during Bloodwise is the leading charity
your diagnosis, treatment and into the research of blood cancers.
beyond, including: They offer support to patients,
• Your haematologist and their family and friends through
healthcare team patient services.
• Your family and friends 020 7504 2200
• Your psychologist (ask your www.bloodwise.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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