Professional Documents
Culture Documents
Leukaemia (CML)
A guide for patients and families
Introduction5
Prognosis18
Treating CML 20
Managing side-effects 27
Nutrition36
Body image 37
Glossary of Terms 42
Acknowledgements
The Leukaemia Foundation gratefully
acknowledges the following groups
who have assisted in the development
and revision of the information
in this booklet:
People who have experienced CML as a
patient or carer, Leukaemia Foundation support
services staff, haematology nursing staff and
clinical haematologists.
4 The Leukaemia Foundation values feedback
from people affected by CML and the health care
professionals working with them. If you would like to
make suggestions, or tell us about your experience
of using this booklet, please contact the Head of
Blood Cancer Support at info@leukaemia.org.au
February 2015
Introduction We have used some medical words
and terms which you may not be
familiar with. Their meaning is explained
This booklet has been written in the booklet and/or in the glossary
of terms at the back of the booklet.
to help you and your family
to understand more about Some of you may require more
information than is contained in this
chronic myloid luekaemia. booklet; we have included some internet
Some of you may be feeling anxious or addresses that you might find useful.
a little overwhelmed if you or someone In addition, many of you will receive
you care for has been diagnosed with written information from the doctors
CML. This is normal. and nurses at your treating hospital.
Perhaps you have already started It is not the intention of this booklet
treatment or you are discussing to recommend any particular form of
treatment to you. You need to discuss 5
different treatment options with
your doctor and your family. your particular circumstances at all
times with your treating doctor and team.
Whatever point you are at, we hope that
the information contained in this booklet Finally, we hope that you find this
is useful in answering some of your booklet useful and we would appreciate
questions. It may raise other questions, any feedback from you so that we
which you should discuss with your can continue to serve you and your
doctor, or specialist nurse. families better in the future.
Accelerated phase
CML is recognised as having Uncommonly, people may present with
three distinct stages or phases: more advanced and rapidly growing
the chronic phase, accelerated CML called accelerated phase. This
phase may also occur in the few people
phase, and blast (crisis) phase. who do not respond well to drug
therapy for chronic phase.
Chronic phase In the accelerated phase, the blood
More than 90% of people are counts become increasingly abnormal
diagnosed in the early chronic phase of and the proportion of blast cells
12 CML. Without treatment the disease increases in the bone marrow and blood.
usually progresses slowly. The
proportion of blast cells in the bone Treatment during the accelerated phase
marrow and blood is low (five% or less). of disease usually involves finding the
Most people are generally well at this most appropriate drug therapy, but may
stage and have few if any troubling sometimes include stem cell
symptoms. In fact, often the diagnosis of transplantation. In patients whose CML
CML is picked up on a routine blood has progressed on drug treatment, a
test performed for another reason. switch to a more effective drug will
usually be required.
Without treatment, chronic phase
usually lasts between three to five
years before progressing to the
more aggressive forms of the disease.
For most patients, however, with
current treatment the duration of the
chronic phase is prolonged indefinitely,
with most expected to have a normal
life expectancy.
Regular blood tests are performed I had a blood test
before starting my new
job. I was shocked to be told I
had CML. I didnt even have
any symptoms.
Blast phase (crisis) In about two thirds of blast phase cases,
CML transforms into a disease
When CML is poorly controlled, it can resembling acute myeloid leukaemia
transform into a very rapidly (AML)*. In the remainder, the
progressing disease resembling acute transformed disease resembles acute
leukaemia. This is known as the blast lymphoblastic leukaemia (ALL)*. In a
phase or blast crisis. The risk of this small number of cases, the blast cells
happening in patients with chronic are said to be undifferentiated or mixed.
phase CML overall is generally less than
5%; it is less than 1% in those with an Information regarding the type of blast
excellent response to drug therapy. cell involved is important because it helps
to guide decisions regarding the most
Very rarely patients may present with effective treatment for your disease.
blast phase disease, without knowing
that they might have had chronic phase Treatment during blast phase of disease
CML previously. is intensive and usually involves
intravenous chemotherapy in 13
Blast crisis is characterised by a rapid combination with the most appropriate
increase in the number of blast cells in the specific CML drug (see later). If this is
bone marrow and blood (usually 30% or successful, some younger patients may
more) and by the development of more then be considered for a bone marrow
severe symptoms. transplant from a donor.
Normal blood cell production is
impaired and severe shortages of * The Leukaemia Foundation has separate
normal blood cells leads to an booklets called: Acute myeloid leukaemia (AML)
increased susceptibility to bleeding, - A guide for patients and families and Acute
infections and anaemia. Blast cells may lymphoblastic leukaemia (ALL) - A guide for
accumulate in various parts of the body patients and families
including the spleen, which can become
rapidly enlarged, the lymph nodes, skin
and central nervous system (brain and
spinal cord).
It used to really bother me that
I didnt know how I got my
CML. I guess I will never
know and Im finally ok
with that.
What causes
CML?
Like other types of leukaemia,
CML is thought to arise from
the chance development of a No-one knows exactly what causes the
mutation (or change) in one CML mutation to occur in most patients.
or more of the genes that In the vast majority of cases it is a
normally control the growth chance, unlucky event. Rarely, excessive
exposure to a chemical called benzene
and development of blood cells. or to very high doses of radiation may be
Within a cell the genes, which control all involved; the risk was increased in
14
aspects of cell growth and survival, are survivors of Hiroshima and Chernobyl.
located on chromosomes within part of The Ph chromosome is found only in
the cell called the nucleus. Each nucleus blood cells and bone marrow cells; it is
contains 46 chromosomes. Almost all not found in eggs or sperm, nor in saliva.
people with CML have a distinctive It is not passed down from parent to child
genetic abnormality known as the and is not contagious.
Philadelphia (Ph) chromosome.
This is an abnormal chromosome formed
when part of chromosome 9 (the abl
gene) breaks off and attaches itself to
part of chromosome 22 (the bcr gene) in
a process known as translocation.
This translocation t(9;22) produces an
overactive, abnormal enzyme (part of a
group of enzymes called tyrosine
kinases) called bcr-abl. This signals the
cell to divide repeatedly, leading to an
excess of leukaemic cells in the blood
and bone marrow.
What are In CML the spleen enlarges as the
leukaemic cells grow within it.
diagnosed?
A bone marrow examination (also called
a marrow biopsy) involves taking a
sample of bone marrow, usually from
the bone at the back of the pelvis
Blood test (called the iliac crest) and sending it to
CML is usually first suspected when a the laboratory for examination under
blood test shows a high white cell the microscope.
count. There are various types of white The bone marrow examination is
cells, but the pattern of which white generally performed in a day procedure
cells are increased usually leads to the unit. A mild sedative and a pain-killer is
suspicion of CML vs other blood given beforehand and the skin is
diseases. Usually the GP who discovers numbed using a local anaesthetic given
this then refers the person to a blood as an injection under the skin.
16 specialist called a haematologist.
After allowing time for the local
As well as a high white cell count, the anaesthetic to work, a needle is
blood test may show anaemia (low red inserted through the skin and outer
blood cells) or a higher than normal layer of bone into the bone marrow
number of platelets. Platelets help the cavity. A syringe is attached to the end
blood to clot but in CML may not of the needle and a small sample of
function properly, increasing the risk of bone marrow fluid is drawn out - this is
easy bruising and bleeding. known as a bone marrow aspirate.
If the results of your blood tests suggest Then the needle is used to obtain a
CML, a small sample of bone marrow small core of bone marrow which will
will need to be examined to help provide more detailed information
confirm the diagnosis and to provide about the structure of the bone marrow
important additional information about and bone - this is known as a bone
the disease. marrow trephine.
Because of the sedation, it is A different technique called
recommended that patients should be polymerase chain reaction (PCR) can
taken home by a family member or detect presence of the bcr-abl gene.
friend. A small dressing over the biopsy This test is also used to assess the
site can be removed the next day. There response to therapy as it can measure
may be some mild bruising or levels of bcr-abl in the blood.
discomfort, which usually is managed
effectively by paracetamol. More Other tests
serious complications such as bleeding Other tests are usually done at diagnosis
or infection are very rare. to provide information about the patients
general health including the functioning
Bone marrow analysis: of kidneys, liver and other vital organs.
cytogenetic and molecular These may include a combination of
blood tests and a chest x-ray and an
genetic tests ECG. These tests are important because
The marrow in CML is filled with large 17
they help in selecting the best treatment
numbers of mature and immature white for you.They can also be compared with
cells and platelets; the diagnosis is later results to assess how the treatment
confirmed by detection of the Ph is tolerated.
chromosome or the bcr-abl gene in the
bone marrow cells. Waiting around for tests can be both
stressful and boring. Remember to ask
Detection of the Ph chromosome is beforehand how long the test will take
performed in a specialised cytogenetic and what to expect afterwards. You
laboratory. Cytogenetic tests provide might like to bring a book, some music,
information about the genetic make-up or a friend for company and support.
of the leukaemic cells, in other words,
the number, structure and abnormalities
in the chromosomes present.
Prognosis The likelihood of achieving the desired
response to treatment (i.e. very low bcr-
abl levels) has been closely correlated to
A prognosis is an estimate of the Sokal score i.e. more people in the
low risk group (with a low score) will
the likely course of a disease. It achieve excellent responses to treatment
provides some guide regarding than those in the high-risk group.
the chances of curing the Having said that, the Sokal score is not
disease or controlling it for a entirely predictive as even in the high risk
given time. group some individuals will respond very
well. In fact, the most important thing is
If you have CML your overall prognosis how well the CML responds to treatment
will depend on a number of factors. based on bcr-abl in blood tests.
These include how well you are
physically, the nature of your disease at An ideal response is a bcr-abl less than
18 10% at 3 months, less than 1% at 6
diagnosis and, most importantly, how
well your disease responds to treatment. months and less than 0.1% at 12 months.
At the very least, it is essential that all
Your doctor is the best person to give patients achieve a bcr-abl less than 10%
you an accurate prognosis regarding by 6 months and less than 1% by 12
your leukaemia as he or she has all months, values associated with a low
the necessary information to make risk of disease progression. If initial drug
this assessment. therapy does not achieve these
The Sokal scoring system provides an responses, then changes may be made
initial estimate of the likelihood of CML in to ensure that you are receiving the
chronic phase to respond well to therapy. best possible treatment at all times for
This system takes different prognostic your particular situation.
factors into account including age,
spleen size, platelet and peripheral
blood blast cell count at diagnosis.
These factors are given individual
scores, which are then tallied to give an
overall score. Depending on the score, Meeting with others
you are regarded as being in either the
low, intermediate or high-risk group. going through similar
issues helps make it more of a
shared experience rather than
just an individual struggle.
Responding to treatment: undetectable levels of bcr-abl had been
offered participation in clinical studies to
commonly used terms investigate whether treatment can be
Complete cytogenetic response (CCR) stopped under medical supervision and
intensive monitoring. Observations over
The Ph chromosome cannot be
a longer period of these patients are
detected using standard laboratory
needed, but in some of these patients
tests. Bcr-abl is usually less than, or
CML has not reappeared after a number
equal to, 1% at this point.
of years and they may be cured
Major molecular response (MMR) Ceasing medications should only be
Bcr-abl value less than or equal to, 0.1%. attempted as part of a clinical trial or
We aim to reach this level as early as with close medical monitoring, as more
possible because we know that once frequent monitoring of your disease is
this level is achieved and maintained, required during this time.
the likelihood of progressing into a 19
more serious form of CML such as Mutations
accelerated phase or blast crisis is Development of a mutation is suspected
minimal. Patients who achieve MMR are when the CML starts to lose response to
largely protected against progression to therapy, as evidenced by a rising bcr-abl.
accelerated or blastic phases, with very These mutations can be detected by
rare exceptions. specialised molecular test called mutation
analysis. This may show mutations that
Complete molecular response (CMR) cause resistance to one drug but not
This refers to the situation when no another. Switching treatment is often
bcr-abl can be detected i.e. the value is needed based on the results of this test.
0%. This does not necessarily mean, An alternative cause of rising bcr-abl is
however, that the CML has completely not taking the medication regularly at
disappeared as there may still be some the prescribed dose; in reality non-
residual disease in very small amounts adherence is the most common cause
which the test is not able to detect. of loss of control of the disease. Non-
adherence increases the risk of
Treatment-free remission or cure
developing a mutation.
This means that there is no evidence of
leukaemia after a period of ceasing
CML therapy. Some patients who have
achieved and consistently maintained
(usually for a minimum of two years)
Treating CML Whilst these drugs are usually very
effective at controlling the disease, most
people are required to take these
The treatment chosen for your medicines for life in order to keep the
disease under control. Only a minority of
CML largely depends on the patients may be cured by TKI therapy and
phase of your disease and your are able to safely stop taking them. People
general health. with well controlled CML are expected to
have a normal life expectancy.
Virtually all people with CML will be
treated with drugs called tyrosine In a very small number of patients, a
kinase inhibitors or TKIs. A tyrosine stem cell transplant (often called a bone
kinase is an enzyme that sends signals marrow transplant) from a matched
to enhance cell growth. As mentioned, donor may be considered. This is
the bcr-abl enzyme is overactive in CML usually only considered in patients who
20 and leads to excessive growth of do not respond well to TKI therapy and
malignant CML cells. TKIs works by have progressive CML. This treatment,
blocking the activity of bcr-abl, thereby while offering the prospect of cure,
preventing the growth and proliferation carries serious risks and requires a
of leukaemic cells. suitably matched donor, hence it is not
used as a first choice therapy.
The three TKIs in Australia, funded on
the PBS are imatinib, (Glivec), nilotinib Promising new and experimental
(Tasigna) and dasatinib (Sprycel). treatments are being developed for
These drugs predominantly inhibit CML all the time. Some of these
cancerous cells that have the Ph treatments are currently being used in
chromosome and have minimal effects clinical trials in Australia and other parts
on normal blood cells. of the world. Your doctor will be able to
discuss with you all of the treatment
The most common decision to be made options suitable for you.
at diagnosis is which of the three
available TKI drugs is most suited to
you. This will vary from person to
person and haematologists need to
consider all the information available
about an individual patient before
making a recommendation. We are all different.
My doctor had to consider all
other aspects of my health before
deciding on which TKI I should use.
Standard therapy Informed consent
Standard therapy refers to a type of Giving informed consent means that
treatment which is commonly used in you understand and accept the risks
particular types and stages of disease. and benefits of a proposed procedure
It has been tried and tested (in clinical or treatment. It means that you agree
trials) and has proven to be safe and that you have adequate information to
effective in a given situation. make such a decision.
Your informed consent is also required if
Clinical trials you agree to take part in a clinical trial, or if
These trials (also called research studies) information is being collected about you or
test new treatments or old treatments some aspect of your care (data collection).
given in new ways to see if they work If you have any doubts or questions
better. Clinical trials are important regarding any proposed procedure or
because they provide vital information treatment please do not hesitate to talk
about how to improve treatment by 21
to your doctor or nurse again.
achieving better results with fewer side
effects. Clinical trials may give people Chronic phase
access to new therapies not yet funded
by governments. In chronic phase, treatment is aimed at
controlling CML, prolonging this phase
If you are considering taking part in a and delaying or preventing the onset of
clinical trial make sure that you symptoms and complications.
understand the reasons for the trial and
what it involves for you. You also need Initial treatment at diagnosis
to understand the benefits and risks of It takes one to two weeks to get
the trial before you can give your approval to start therapy with a TKI
informed consent. Talk to your doctor drug. In the interim, patients with very
who can guide you in making the best high white cell counts at diagnosis may
decision for you. be given a short course of
chemotherapy tablets called
hydroxyurea to reduce the CML count.
Most people tolerate hydroxyurea very
well. It does not usually cause nausea or
significant hair loss, although it can
cause dry skin or a rash.
Tyrosine kinase inhibitors People who tolerate imatinib well and
achieve a major molecular response are
(TKIs) likely to have a normal life expectancy,
Imatinib (Glivec) although very few patients have
received imatinib for longer than 15
The first clinically available TKI was a
years, so we dont yet know the stability
drug called imatinib mesylate (imatinib).
of the response to this drug beyond this
It made a huge leap forward in the
time. However, there is currently no
effectiveness of how CML was treated
evidence to suggest that these patients
and replaced stem cell transplants as
will not continue to respond to imatinib
the best treatment approach.
beyond this time frame.
Imatinib produces rapid control of the
As imatinib was the first TKI medication
blood count in most patients with
used for people with CML, we know
chronic phase CML and leads to a high
more about its long term use and its
rate (60% after 5 years) of major
22 side effects than the other TKIs. This
molecular responses (i.e. substantial
long term usage data has given
lowering of bcr-abl levels), thus
confidence to doctors and patients and
controlling the disease and reducing
imatinib is therefore currently the most
the probability of progression to blast
commonly used TKI therapy for people
phase for the majority of people.
with CML in Australia.
A minority (approx. 20%) of people with
CML will not respond to imatinib initially Possible side effects
(in the first 3-6 months of treatment) OR Side effects are usually mild and vary
respond to imatinib but lose their from person to person and according to
response (see earlier about mutations) the dose given. They can be unpleasant
and require other therapy. Another but most are temporary and improve
reason for replacing imatinib with with time. It is important that people
another drug is side-effects; this occurs report any side effects as many of them
in approximately 15% of patients. can be treated successfully, reducing
any unnecessary discomfort.
Side effects of imatinib and treatment: 7. a temporary reduction in the number
of white cells, platelets and red cells;
1. nausea and vomiting: this can be
this is a rare side-effect, and is usually
prevented by taking imatinib with the
mild. In severe cases blood
largest meal of the day with a large
transfusions may be required for a
glass of water; imatinib should NOT
few weeks, and it may be necessary
be taken on an empty stomach
to stop imatinib temporarily.
2. fatigue: this is not uncommon and is
Imatinib interacts with many other drugs
often subtle such that patients can
which may interfere with the
continue their normal daily activities.
effectiveness of imatinib by increasing
In some patients, however, it can be
or decreasing its concentration in your
profound and require switching to
blood. These include some prescription
another TKI drug.
and over-the-counter drugs (including
3. fluid retention and swelling: puffiness herbal remedies and St Johns Wort )
around the eyes is common with and grapefruit juice (and fruits from the 23
imatinib. Some patients on imatinib same family). These should be avoided.
put on some weight due to fluid
Your imatinib medication box should
retention. This tends to stabilise after
have an information sheet inserted
a few months and usually does not
please ensure you read this for more
require treatment
information about product safety. It is
4. muscle cramps: these can be a important that people taking imatinib
problem in some patients and may speak to their doctor before using any
require medication, often magnesium other drugs or supplements.
tablets, to reduce the severity
It is recommended that women do not
5. an itchy skin rash: this happens become pregnant while taking imatinib
occasionally and usually responds to as it may affect the developing baby.
topical ointment and temporarily You or your partner should use an
stopping the drug. Persistent rash is effective form of contraception. It is
rarely a reason to permanently stop possible, however, for women with CML
the drug to have children but it is very important
to discuss this with the treating
6. diarrhoea: this may require
haematologist well in advance.
medication such as Gastrostop
In addition, it is not known if the imatinib
(loperamide) to overcome; if severe
passes into the breast milk and hence
(which is uncommon), it may require
breast-feeding is not recommended.
stopping imatinib
Many people with CML have been able to Most people have only mild side-effects
have healthy children. Please ensure you with nilotinib, although some people
speak with your doctor if you are have more troubling side-effects.
considering conceiving to ensure you have The more common side-effects include
the best advice on how to do this safely. fatigue, rash, itching, nausea and
constipation. Less commonly, nilotinib
In men, imatinib may have an effect on
can cause anaemia, a low platelet
sperm count in some cases. Men may
count, or a low white blood cell count.
wish to store sperm prior to commencing
CML treatment. There is no evidence of Nilotinib can cause an abnormal heart
any increased risk of abnormalities in rhythm called QT prolongation in a small
babies fathered by men taking imatinib. It number of people. This risk is increased
is important that you dont stop taking by an imbalance of the electrolytes in
imatinib unless you are instructed to do the blood (low potassium and
so by your doctor. To be effective magnesium levels) and taking other
24 imatinib needs to be taken every day. medications that also cause QT
prolongation. Your doctor will monitor
Nilotinib (Tasigna) this carefully.
Nilotinib has a similar mode of action to Around 1% of people taking nilotinib will
imatinib. It is effective in some people develop pancreatitis, a condition with
who have become resistant to imatinib inflammation and swelling of the
as well as newly-diagnosed patients. pancreas, often accompanied by
People who experience side effects abdominal pain and vomiting. If
from imatinib usually do not have similar pancreatitis is caused by nilotinib a
side effects when taking nilotinib. different TKI should be used.
Nilotinib is a tablet taken in the morning People taking nilotinib also appear to
and evening. It is very important that have a higher risk of blocked arteries
nilotinib is taken on an empty stomach (which may lead to heart attacks and
no food should be eaten for 2 hours strokes), especially those taking higher
before AND 1 hour after taking each dose. doses. In a recent study blocked
arteries occurred in 7.5% in people after
5 years taking nilotinib 300mg twice per
day and 13.4% in patients taking 400mg
twice per day.
The mechanism behind this is not fully Generally dasatinib is well tolerated,
understood. Hence nilotinib may not be although some people have troubling
the preferred TKI medication in patients side-effects. These include diarrhoea,
with other risk factors for heart disease easy bruising and bleeding and fatigue.
and strokes such as diabetes, high The most important side-effect is the
blood pressure and high cholesterol. development of fluid in the space around
the lungs, called a pleural effusion, which
Nilotinib can cause an increase in blood
may lead to a persistent dry cough and
sugar levels on both diabetics and non-
shortness of breath. This side-effect is
diabetic patients and in cholesterol
more common in elderly patients.
levels and these should be monitored
closely whilst taking this drug. If identified early, pleural effusion can
be well managed. This may require
It is recommended that you or your
treatment with a steroid medicine
partner use a suitable form of
(prednisolone), temporarily stopping
contraception while taking nilotinib as
dasatinib (usually with restarting the 25
this drug may harm the developing
medication at a lower dose), or
baby. Mothers are advised not to breast
occasionally a procedure to drain the
feed while taking nilotinib.
fluid from the chest. Those with lung
Dasatinib (Sprycel) disease, or the elderly, should be
monitored closely.
Dasatinib is also a tyrosine kinase
inhibitor, with activity in newly diagnosed Very rarely dasatinib can cause a
disease as well as disease resistant to condition called pulmonary
imatinib. It is a tablet that is swallowed hypertension which can result in excess
once daily and can be taken with or pressure on the heart and symptoms
without a meal. It comes in 100mg, 50mg including shortness of breath. This can
or 20mg tablets. The usual daily dose is sometimes be diagnosed using an
100mg per day, but lower doses may be ultrasound of the heart (called an
effective in older patients. echocardiogram). If detected, dasatinib
needs to be stopped permanently.
It is recommended that you or your
partner use a suitable form of
contraception while taking dasatinib.
Mothers are advised not to breast feed
while taking dasatinib.
Adherence to treatment Some mutations do not respond well to
TKIs, making treatment much more
Adherence, also commonly difficult. It is therefore very important
called compliance, to treatment that you take your medication every
for CML is very important for day, and not make any changes to your
treatment without discussing it first with
the drugs to work effectively. If your haematologist.
there is not enough drug in the
Research has shown that the faster the
body, it is possible that the CML medications reduce bcr-abl levels to
cells may become resistant (via undetectable levels, the better the
a process called mutation). outcome is long term. Therefore
adherence to therapy is particularly
important in the first two years of
treatment. Unfortunately, this is also the
26 time that side-effects are most
prevalent. To get the best out of your
therapy, work closely with your treating
team to manage your side effects, to
best enable you to adhere to the full
dose of medications prescribed.
It can be so tempting to
take a couple of days off
therapy to help me feel a little
better. When I heard what the
consequences can be I decided it
wasnt worth the risk.
Managing side-effects
Remember that no two people are the same. In helping to make the best treatment
decision about any side-effects you may experience, your doctor will consider the
specific details of your situation.
Chemotherapy
Chemotherapy literally means therapy
Interferon alpha with chemicals. Many chemotherapy
Interferon alpha used to be standard drugs are also called cytotoxics (cell toxic)
treatment for CML before imatinib was because they kill cells; especially ones
developed. These days it is not used that multiply quickly like cancer cells.
very often. It may still have a role in a Chemotherapy for CML in chronic
minority of cases (e.g. during phase usually involves hydroxyurea, a
pregnancy), and researchers are drug which can be taken in tablet or
investigating whether it provides extra capsule form at home and has been
benefit when combined with a TKI. found to be very effective at controlling
Interferon alpha is given as a small a high white cell count. On the other
injection under the skin. It can have hand, people in accelerated or blast
significant side effects including flu-like phase CML may benefit from more
31
symptoms - chills, fevers, aches and intensive anti-leukaemia therapy.
pains and weakness. It can also cause If you are having chemotherapy your
other unpleasant symptoms such as doctor and nurse will tell you about the
nausea, loss of appetite and depression. side-effects you might experience and
These symptoms are usually temporary. how they can be best managed.
Your doctor or nurse will explain any side
effects you might experience if you are
having this form of treatment and how
Accelerated and
they can be managed. blast phase
Some people already have advanced
disease at diagnosis, whilst others may
experience disease progression to
accelerated and blast phases. These
are both uncommon.
In these advanced phases, treatment is
aimed at re-establishing the chronic phase
of CML, and reducing any troublesome
symptoms. There are several treatment
options which may be used depending on
your particular circumstances.
Accelerated phase disease: usually Stem cell transplant* (bone
a stronger TKI (e.g. nilotinib or
dasatinib) is preferred marrow transplant)
An allogeneic (donor) stem cell
Blast phase disease: a combination of
transplant currently offers the chance of
a TKI with intensive chemotherapy is
curing CML but has significant side-
usually necessary. This commonly
effects in many patients. It is generally
involves the use of a combination of
now used only for people in whom the
chemotherapy drugs given
CML has not responded well to TKI
intravenously (into a vein).
therapy or for those rare patients who
The drugs chosen are tailored to treat present with or develop accelerated or
the type of leukaemic transformation blast phase disease. Around 10 people
which has occurred (acute myeloid will receive a stem cell transplant for
leukaemia (AML) or acute lymphoblastic CML in Australia each year.
leukaemia (ALL)). This treatment is given
32 A transplant involves giving very high
in hospital and the side-effects can be
doses of chemotherapy, sometimes in
more severe.
combination with radiotherapy, in an
Not everyone is suitable for this form of attempt to completely destroy the
treatment, especially if they are elderly abnormal stem cells in your bone marrow.
or not well enough to tolerate the
These cells are then replaced with
potential side effects. In younger, fitter
healthy stem cells which have been
patients a stem cell transplant may be
donated, usually from a brother or sister
necessary. Treatments to reduce
who has the same tissue type as yours.
symptoms of CML may include blood
In some cases the donor is not a family
transfusions, antibiotics and other drugs
member, but has a similarly matched
to help keep you as well and comfortable
tissue type. This type of transplant is
as possible during this time.
called a matched unrelated donor
transplant (MUD) or volunteer unrelated
donor transplant (VUD).
* The Leukaemia Foundation has a separate
booklet called: Allogeneic Transplants - A guide
for patients and families.
How do I know In general, bcr-abl values are then
tested every 3-4 months for the rest of
therapies
Serious infections occur very rarely in
people with CML. Dont hesitate to
contact your doctor or hospital if you
develop any of the following signs of
Supportive care plays an infection so that you can be treated
important role in the appropriately, with antibiotics and other
treatment of many people drugs if necessary:
with CML. This involves a temperature of 38oC and/or an
making every effort to episode of shivering (where you
shake uncontrollably)
improve your quality of life,
by relieving any symptoms coughing or shortness of breath with
a fever
you might have and by
35
preventing and treating any a sore throat and/or a head cold
complications that arise from passing urine frequently or a stinging
your disease or treatment. pain when passing urine
if you are feeling generally unwell.
Blood transfusions, antibiotics, careful
attention to side-effects and A number of antibiotics can interact with
psychological support and appropriate CML medications so its important that
use of complementary therapies, are all your GP knows what CML medication
important elements of supportive care. you are taking in order to check for any
potential interactions before prescribing
an antibiotic.
Other indications to see your doctor in Nutrition
addition to routine appointments:
A healthy and nutritious diet is important
If you are bleeding (for example blood in helping your body to cope with your
in your urine, stools, sputum, bleeding disease and treatment. Talk to your
gums or a persistent nose bleed) or doctor or nurse if you have any
bruising very easily questions about your diet or if you are
If any surgery is planned. Advice may considering making any radical changes
be required from your haematologist to to the way you eat. You may wish to see
ensure that the surgery is completed a nutritionist or dietician who can advise
successfully without problems due to you on planning a balanced and
your disease or its treatment nutritious diet.
If you are thinking about using herbs or
Complementary therapies vitamins it is very important to talk this over
Complementary therapies are therapies with your doctor first. Some of these
36
which are not considered standard substances can interfere with the
medical therapies. Many people effectiveness of treatments you are having.
however find that they are helpful in People with CML should avoid
coping with their treatment and recovery grapefruit as it can interact with TKIs.
from disease. There are many different
types of complementary therapies such
as yoga, exercise, meditation, prayer,
acupuncture and relaxation.
Complementary therapies should
complement or assist with
recommended medical treatment for
CML. They should not be used instead
of, or as an alternative to, medical
treatment. It is important to realise that
no complementary or alternative
treatment alone has proven to be
effective against CML. It is also
important that you inform your doctor if
you are using any complementary or
alternative drug therapies, or remedies,
in case they cause any problems with
your disease, or its medical treatment.
Body image
It is likely that the diagnosis and treatment
of CML will have some impact on how you
feel about yourself as a man or a woman
and as a sexual being.
41
Glossary of Alopecia
Hair loss. This is a side effect of some
Terms kinds of chemotherapy and
radiotherapy. It is usually temporary.
Cytogenetic tests
Genes
The study of the genetic make-up of the
Collections of DNA. Genes direct the
cells, in other words, the structure and
activity of cells. They are responsible for
number of chromosomes present.
the inherited characteristics that
Cytogenetic tests are commonly carried
distinguish one individual from another.
out on samples of blood and bone marrow
Each person has an estimated 100,000
to detect chromosomal abnormalities
separate genes.
associated with disease. This information
helps in the diagnosis and selection of the
most appropriate treatment.
Granulocytes High dose therapy
A family of white blood cells that The use of higher than normal doses of
contains granules in their cytoplasm chemotherapy to kill off resistant and/or
(neutrophils, eosinophils and basophils). residual (left over) cancer cells that have
They protect the body by seeking out survived standard-dose therapy.
and destroying microorganisms.
Imatinib mesylate (Gleevec or
Growth factors and cytokines Glivec)
A complex family of proteins produced by A relatively new drug used to treat chronic
the body to control the growth, division myeloid leukaemia and other Philadelphia
and maturation of blood cells by the bone chromosome positive (Ph+) leukaemias.
marrow. Some are now available as drugs Imatinib is classified as a tyrosine kinase
as a result of genetic engineering and inhibitor. It works by targeting the
may be used to stimulate normal blood abnormal bcr-abl gene thereby blocking
cell production following chemotherapy the leukaemia-causing effects of the
or bone marrow or peripheral blood stem enzyme tyrosine kinase. Also known as
cell transplantation. imatinib (Gleevec or Glivec).
Oncologist
General term used for a specialist doctor
who treats cancer by different means, e.g.
medical, radiation, surgical oncologist.
Pathologist Prognosis
A doctor who specialises in the An estimate of the likely course of
laboratory diagnosis of disease, and a disease.
how disease is affecting the organs of
the body. Purpura
Purple spots on the skin, often
Peripheral blood stem cell collection accompanied by bleeding from the
The collection of stem cells from the gums. It is caused by a shortage of
circulating blood stream. platelets as well as fragile skin.
Translocation
A chromosomal abnormality in which
part of the one chromosome is
transferred to another.
T-cell
A type of white cell involved in
controlling immune reactions.
Notes
51
Notes
52
Making a donation
The Leukaemia Foundation is the only national not-for-profit organisation
dedicated to the care and cure of patients and families living with leukaemia,
lymphoma, myeloma and related blood disorders. The Foundation receives
no ongoing government support and relies on the generosity of the
community to support our Vision to Cure and Mission to Care.
Name
Address
Postcode
Phone Mobile
Email
53
Cardholders Name
Signature
Your privacy is important to us. That is why we treat your personal information with confidence.
To learn more about how and why we collect and use any personal or sensitive information about you,
please view our Notification Statement at www.leukaemia.org.au/privacy
Please send me a copy of the following booklets:
Acute Lymphoblastic
Leukaemia in Children (ALL) Books for children:
Name
Address
Postcode
Phone Mobile
POST TO The Leukaemia Foundation, Reply Paid 9954 in your capital city
55
This information booklet is produced
by the Leukaemia Foundation and is one in
a series on leukaemia, lymphoma, myeloma,
MDS, MPN and related blood disorders.
Copies of this booklet can be obtained
from the Leukaemia Foundation
in your state by contacting us.
The Leukaemia Foundation is a not-for-profit
organisation that depends on donations
and support from the community.
Please support our work.
February 2015
Contact us
1800 620 420
info@leukaemia.org.au
leukaemia.org.au