Relapse in Chronic Lymphocytic Leukaemia (CLL) - A Guide for Patients
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Relapse in Chronic Lymphocytic Leukaemia (CLL) A Guide for Patients
Introduction
A relapse is the return of leukaemia after treatment.
Specifically, this booklet is about a relapse in chronic
lymphocytic leukaemia (CLL).
The booklet was written by our
Patient Information Writer,
Isabelle Leach, and peer reviewed
by Helen Knight, CLL Clinical
Nurse Specialist at the Centre
for Clinical Haematology in
Nottingham University Hospitals.
We are also grateful to our patient
reviewer, Amanda Salter, for their
contribution.
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: 05/2019
2 www.leukaemiacare.org.uk Review date: 05/2021In this booklet
Introduction 2
In this booklet 3
About Leukaemia Care 4
What is Chronic Lymphocytic Leukaemia? 6
What is relapsed CLL? 8
Symptoms and diagnosis of relapsed CLL 12
How is relapsed CLL treated? 14
Seeing your doctor 24
Telling your family 26
Managing your emotions 28
Survivorship 32
Palliative care 34
End of life care 36
Glossary 38
Useful contacts and further support 43
Helpline freephone 08088 010 444 3About Leukaemia Care
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right information, advice and support.
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Helpline freephone 08088 010 444 5What is Chronic Lymphocytic
Leukaemia?
In chronic lymphocytic leukaemia lymphatic vessels, which run
(CLL), the lymphocytes in the adjacent to the small blood
bone marrow start multiplying vessels in the body, drain lymph
excessively leading to large fluid from all over the body to
numbers of abnormal, immature clear excess fluid and act as
cells called blasts, which prevent a blood filter helping to fight
the bone marrow from producing infection with the help of the
enough healthy blood cells of all B-cells and T-cells.
types. Lymphocytes are a type of
CLL is the most common form of
white blood cell involved in the
leukaemia in adults in Western
immune response.
countries. Approximately 3,500
There are three types of adults are diagnosed with CLL
lymphocytes: each year in the UK alone, which
is equivalent to 10 new cases
1. B-cells produce antibodies every day. Slightly more men than
that seek out and immobilise
women tend to be affected by CLL
bacteria, viruses, and toxins
and it is often diagnosed in older
which invade the body.
people, being diagnosed in 59% of
2. T-cells destroy the invading people aged 70 years and over. For
organisms that have been reasons that are not understood,
tagged by the B-cells as well CLL is more common in White
as cells that have become people and less common in Asian
cancerous. populations. The disease is rarely,
if ever, seen in children.
3. Natural killer (NK)-cells attack
cancer cells and viruses. Full details of the characteristics,
diagnosis and treatment of
All these types of white blood CLL are given in the Patient
cell are found in the blood, bone Information Booklet titled
marrow, and lymphatic system. chronic lymphocytic leukaemia
The lymphatic system is a part (CLL), which is available on
of the immune and circulatory the Leukaemia Care website at
systems. A network of small
6 www.leukaemiacare.org.ukhttps://www.leukaemiacare.org.
uk/support-and-information/
help-and-resources/
information-booklets/
In this booklet, relapse in CLL will
be examined.
Helpline freephone 08088 010 444 7What is relapsed CLL?
In patients with CLL who require ••Blood and bone marrow at
treatment, the mainstay the cell level (cytogenetic
of the treatment is chemo remission): Diseased cells
immunotherapy, which consists have been greatly reduced, or
of chemotherapy to which no longer appear, in the bone
immunotherapy is added. Where marrow.
the CLL cells have a particular
chromosome abnormality, ••Blood and marrow at the
molecule level using sensitive
such as a 17p deletion or TP53
tests such as polymerase
mutation, a novel agent such
chain reaction analysis or
as ibrutinib is used instead of
flow cytometry (molecular
chemotherapy.
remission): No evidence of
The nature of CLL as a chronic disease in the blood cells and/
disease means that it is not or bone marrow is seen at all.
a curable disease, and that a
relapse is expected to happen Why does relapse
at some point. A relapse is when happen?
a patient initially responds to
While the recent treatment of
leukaemia therapy but, after
CLL with chemo-immunotherapy
six months or more, response
has resulted in improved patient
to treatment stops. This is also
responses and overall survival
sometimes called a recurrence.
compared to chemotherapy alone,
Refractory CLL occurs when the
a sizeable number of patients will
cancer has not responded to first-
still relapse and need subsequent
line treatment (first treatment
treatment.
given).
Relapse is often linked to the
Remission can be established by
cause of the CLL, particularly
examining:
when chromosome mutations
••Blood samples are involved. These genetic
(haematological remission): characteristics are useful for
Blood counts return within predicting how the CLL will
normal ranges. progress, and which patients will
8 www.leukaemiacare.org.ukrespond to therapy or relapse. with standard CLL patients
and patients with lower-risk
For patients with CLL, analysis
chromosomal abnormalities.
of chromosomes has detected
several reoccurring mutations: Another indication of a reduced
survival rate in CLL is an
•• 13q deletion - 55% of patients unmutated immunoglobulin
•• 11q deletion - 18% of patients variable region heavy chain (IgVH)
gene. This gene is responsible
•• 17p deletion - 7% of patients for generating antibodies used
previously untreated and 30% of by the immune response. IgVH
patients who relapsed mutation status has identified
•• Trisomy 12 (3 copies of two subtypes of CLL that have a
differing clinical course:
chromosome 12) - 16% of
patients 1. Patients with a mutated IgVH
Patients with 13q deletion tend have a subtype of CLL which is
to have good prognoses and slow to progress and with little
patients with trisomy 12 have an symptoms. These patients have
intermediate prognosis. Therefore, a better prognosis.
these patients may be more 2. Patients without a mutated
fortunate in avoiding relapses. IgVH have an aggressive
Patients with 11q deletion and subtype of CLL with a poorer
17p deletion are known to have a prognosis, a shorter duration
shorter time before relapse where of complete remission and a
further treatment will be required. greater chance of relapse.
Deletion of chromosomal region The mutation status of IgVH can
17p13 results in the loss of one be detected by DNA sequencing.
of the variants of the TP53 gene. DNA sequencing involves
This abnormality is predictive of determining the exact order of
a poor response to treatment and the four organic molecules (also
is associated with the shortest called nucleotides: adenine,
time before relapse compared guanine, cytosine, and thymine)
Helpline freephone 08088 010 444 9What is relapsed CLL? (cont.)
which make up a molecule of DNA. first line treatment is around five
years. When it has been identified
How often does relapse that the disease has come back,
occur? further treatment options will be
discussed with you by your doctor.
Despite the encouraging
Details of these are given in the
responses in patients with
section ‘How is relapsed CLL
CLL who have received chemo-
treated?’
immunotherapy, most patients
will relapse at some point within
the first five years of starting
treatment.
The first-line chemo-
immunotherapy regimen of FCR
(combination of fludarabine,
cyclophosphamide and
rituximab) is most frequently
used in patients with CLL, as
long as they are fit and well with
little or no other health problems.
However, even after this treatment
regimen, approximately 6% of
patients will relapse within six to
12 months and another 14% will do
so within two years.
Patients whose first remission
lasts less than three years have
a short survival period, whatever
treatment they were given
subsequently. For patients with
a first remission greater than
three years, a number of other
treatments are available. The
average remission after FCR as a
10 www.leukaemiacare.org.ukHelpline freephone 08088 010 444 11
Symptoms and diagnosis of
relapsed CLL
What are the •• Bruising and bleeding due to
a lack of platelets in the blood
symptoms of relapsed (thrombocytopenia)
CLL?
The course of CLL can be very
•• Swollen lymph nodes in the
neck, armpits or groin
varied. CLL usually develops
extremely slowly, and more than •• Swollen abdomen, abdominal
half of all patients do not have discomfort, inability to eat large
any symptoms in the early stages meals caused by an enlarged
of the disease. Over time, CLL spleen or lymph nodes
patients often develop symptoms
as a result of lower than normal •• Changes in appetite
numbers of red blood cells •• Weight loss
(anaemia), white blood cells
(neutropenia) and/or platelets How is relapsed CLL
(thrombocytopenia). diagnosed?
In patients with CLL who relapse, The majority of relapses in
the symptoms and signs that patients with CLL are diagnosed
may occur are similar to those for relatively early on because after
newly diagnosed CLL, and include: first-line or second-line treatment,
patients are monitored regularly.
•• Feeling tired all the time The diagnosis of relapsed CLL
(fatigue)
is similar to that of the initial
•• Infections – these may be diagnosis.
frequent, persistent and/or
Diagnosis of CLL requires the
severe (due to neutropenia)
presence of increased levels
•• A high temperature (fever) (≥5000 per mm3) of monoclonal
(genetically identical) B-cells
•• Severe sweating at night in the blood for at least three
•• Breathlessness, tiredness and months. The clonal nature of
the circulating B-cells should
headaches due to a lack of red
blood cells (anaemia) be confirmed by flow cytometry
(a test that identifies specific
12 www.leukaemiacare.org.uksurface markers on the cell). of proteins on the surface of the
lymphoblast cells
Diagnosis is achieved using the
following tests: Immunophenotyping
Complete blood count Immunophenotyping is used
primarily to help diagnose
Detects the number of red blood
and classify leukaemias and
cells, white blood cells and
lymphomas, and guide their
platelets.
treatment. Where possible,
Peripheral blood smear it may be able to predict the
aggressiveness of the leukaemia
Blood sample is viewed under
and its responsiveness to certain
a microscope to count different
treatment.
circulating blood cells, and see
whether the cells look normal. Markers detected on the cells will
help characterise any abnormal
Bone marrow aspiration and cells present. In conjunction with
biopsy the patient’s clinical history,
The aspiration procedure removes physical examination, signs and
a liquid marrow sample and the symptoms, and laboratory tests,
biopsy removes a small amount immunophenotyping can help
of bone filled with marrow. make a diagnosis.
Medication is given to numb the
Immunophenotyping is routinely
area, or a general anaesthetic is
performed by flow cytometry
performed, in order to remove a
which processes either blood,
sample from the hip bone. These
bone marrow fluid or tissue by
samples can be examined for:
adding specific antibodies that
•• Percentage of lymphoblasts in have been tagged with fluorescent
the bone marrow markers. These antibodies, also
called cell markers, bind to
•• Any abnormalities of the corresponding antigens on the
lymphocytes lymphocytes. The flow cytometer
•• Immunophenotyping: This rapidly measures the size and
procedure identifies the types internal cellular structures
Helpline freephone 08088 010 444 13Symptoms and diagnosis of
relapsed CLL (cont.)
of thousands of cells, and benefit from:
assesses the type and quantity
of fluorescent antigen-antibody ••Lumbar puncture - This is used
to determine if the lymphoblast
complexes present. For these
cells are in your central nervous
practical reasons, flow cytometry
system (brain and spinal cord).
is preferred to immunochemistry
for immunophenotyping. ••Imaging tests – Ultrasound
and computed tomography
Chromosomal or cytogenetic
scanning to accurately detect
analysis
the enlarged lymph nodes,
•• Blood smear samples can be liver and spleen before starting
used to identify certain changes treatment.
in the number and size of
chromosomes within cells that ••Lymph node biopsy – This is
might have led to the relapse. carried out when lymph nodes
are swollen. A lymph node
•• This can be achieved easily and biopsy is a minor surgical
accurately with fluorescent procedure where a small sample
in situ hybridisation (FISH) is taken from a lymph node
which uses fluorescent dyes and then examined under a
to attach to certain parts of microscope. This is usually
chromosomes. FISH analysis performed as a day case and
should always be carried out does not require a hospital stay.
prior to a patient receiving
treatment as it can determine ••Staging - This is a grading
types of leukaemia likely to system used by consultants to
relapse or their response to describe the extent of the CLL,
treatment. in terms of location, its effect
on the blood count, and the
The following tests and number and size of existing
procedures are carried out to lymph nodes. Grading CLL helps
confirm the diagnosis, confirm your doctor predict how quickly
the stage of the CLL, and enable the cancer may grow and
your consultant to determine the spread, as well as decide the
treatment you are most likely to best treatment for you. There are
14 www.leukaemiacare.org.uktwo main systems used to stage
CLL. Most doctors in the UK and
Europe use the Binet system,
whereas in the USA doctors
more commonly use the Rai
system.
Helpline freephone 08088 010 444 15How is relapsed CLL treated?
To date, the FCR treatment response obtained with previous
regimen has given the best treatment, length of the response,
complete remission rate, longest and their characteristics at the
duration of remission, and time of relapse.
the most extensive survival
for patients with CLL. The Alternative treatment
regular chemo-immunotherapy options
regimens for the treatment of
Most patients with relapsed or
patients with CLL are FCR or the
refractory CLL require second-line
combination of bendamustine
therapy (treatment other than the
and rituximab. However, with
type used the first time around).
both these regimens, patients
Second-line drug regimens may
may experience significant side
include:
effects, especially older patients
or those with other health issues. •• Chlorambucil with a monoclonal
antibody (if the patient only
Most patients who respond to
received chlorambucil on its
treatment will eventually relapse
own as first line therapy)
and require second-line therapy
(treatment other than the type •• Targeted therapy: ibrutinib or
used the first time around). idelalisib in combination with
However, patients who are in rituximab (when the disease
remission for less than three has been treated but relapsed
years the first time have shorter within 24 months)
periods of remission and shorter
intervals between treatments, •• Venetoclax
whatever treatment regimen they •• Clinical trials may also be
were given subsequently. a possibility if suitable and
The most appropriate treatment available
for relapsed patients depends
largely on the characteristics of
their CLL, and is also dependent
on their prior therapies, best
16 www.leukaemiacare.org.ukChlorambucil with a Ibrutinib is an inhibitor of
monoclonal antibody Bruton’s tyrosine kinase, and
(chlorambucil chemo- idelalisib is a selective inhibitor
immunotherapy) of the delta phosphatidylinositol
3-kinase enzyme. Both these
If patients only received
targeted therapies inhibit specific
chlorambucil as first-line
signalling pathways for B-cells.
therapy, then chlorambucil with
a monoclonal antibody can be an Idelalisib has regulatory approval,
alternative treatment for patients when used in combination with
who relapsed. Chlorambucil is a rituximab, in previously-treated
type of chemotherapy called an CLL and CLL with 17p deletion or
alkylating agent which interferes TP53 mutation that cannot be
with DNA and stops cancer cells treated with any other therapy.
proliferating. It is approved by the National
Institute for Health and Clinical
When combined with other
Excellence (NICE).
monoclonal antibody drugs such
as ofatumumab or obinutuzumab, Ibrutinib has regulatory approval
chlorambucil was superior to for previously-treated CLL and
chlorambucil alone in two large untreated CLL with 17p deletion
phase 3 studies. or TP53 mutation, and is also
approved by NICE.
Targeted therapy
Both ibrutinib and idelalisib are
Patients with 17p deletions
effective in patients with high-
or TP53 mutations, for whom
risk features and are able to
chemo-immunotherapies are
control CLL in difficult-to-treat
not suitable, can be treated with
patients better than currently
the targeted therapies B-cell
approved therapies. This makes
receptors inhibitors such as
them the drugs of choice for CLL
ibrutinib and idelalisib, or B-cell
patients with TP53 mutations
lymphoma-2 inhibitors such as
and patients resistant to chemo-
venetoclax.
immunotherapy. Both idelalisib
Helpline freephone 08088 010 444 17How is relapsed CLL treated?
(cont.)
and ibrutinib in combination Ibrutinib
with monoclonal antibodies have
The approval of ibrutinib as
improved survival in relapsed/
initial therapy for CLL patients
refractory CLL.
was based on the phase 3
Idelalisib in combination with RESONATE 2 trial in which 269
rituximab previously untreated CLL patients
>65 years received ibrutinib or
Idelalisib is currently only
chlorambucil. After a follow-up
approved for relapsed/
period of 18.4 months, patients
refractory CLL when combined
on ibrutinib showed a significant
with rituximab, based on the
increase in overall survival with
pivotal phase 3 trial which
an estimated survival rate at 24
compared treatment with the
months. Overall response rate
combination against rituximab
was also significantly improved
alone. In this randomised trial,
with ibrutinib compared with
the investigators recruited 222
chlorambucil. Complete remission
patients >65 years, who had been
was achieved by five patients
treated with first-line treatment
receiving ibrutinib compared
but relapsed within 24 months,
with two patients receiving
and had decreased renal function
chlorambucil.
as well as previous therapy-
induced myelosuppression. Positive responses with idelalisib
Treatment with the combination and ibrutinib are increasingly
of idelalisib and rituximab, when being reported in patients with
compared with rituximab alone, relapsed/resistant CLL. However,
significantly improved overall for a number of patients with
response and overall survival at 12 relapsed CLL, who were classified
months in patients with relapsed as high-risk, these therapies do
CLL who were less able to undergo not achieve a long-term response.
chemotherapy. The clinical benefit
of this drug was also present in Venetoclax
the high-risk patients with del Venetoclax is an inhibitor of
(17p) and/or TP53 mutation. the activity of a protein called
18 www.leukaemiacare.org.ukB-cell lymphoma-2. This protein a complete response achieved in
regulates the natural death 20%.
of cells, particularly cancer
cells. In the United Kingdom, Rechallenge with first-
venetoclax has conditional line treatments
approval for CLL when other
Patients with CLL who have
treatments have failed or are
relapsed can be re-challenged
unsuitable. Conditional approval
with the first-line treatments
for marketing authorisation
of FCR or the combination of
(available for prescription) was
bendamustine and rituximab, but
granted because additional data
this is extremely rare.
is being considered; however, the
use of venetoclax in the interest FCR
of public health and the benefit of
For patients without the high-risk
immediate availability outweighs
features for being refractory to
the risk from less comprehensive
therapy or having chromosome
data than normally required.
17 abnormalities, FCR is an
NICE recommends the use of effective and safe therapy. For
venetoclax in patients with a 17p high-risk and elderly patients,
deletion or TP53 mutation, and optimal treatments still need to
whose disease has progressed be defined.
despite treatment with
Bendamustine and rituximab
chemo‑immunotherapy and a
combination
B‑cell receptor inhibitor.
For the many CLL patients >65
In a phase 1 study to determine years who experience severe
the best dose of venetoclax to toxicity with FCR (because
use in safety and efficacy trials of the serious infections and
(dose-escalation study), the suppression of the bone marrow),
overall response rate across bendamustine combined with
all doses of 116 patients who rituximab (BR) is commonly used
received venetoclax was 79% with as it is better tolerated.
Helpline freephone 08088 010 444 19How is relapsed CLL treated?
(cont.)
Allogeneic stem cell does not respond to two lines
of treatment and shows an
transplantation objective response to B-cell
The treatment options described receptor inhibitors or to
previously may render allogeneic treatment in a clinical trial
stem cell transplantation (ASCT)
outmoded because patients •• CLL which does not show
can now be maintained in a an objective response or
prolonged disease-free state progresses after B-cell receptor
with these treatments, even if inhibitors, regardless of whether
they are not curative. However, an objective response is
especially for young healthy achieved
patients, ASCT remains the only
potentially curative option if
Supportive therapy
they are fortunate enough to Supportive therapy or palliative
have a suitable donor. ASCT is care is valuable during treatment
rarely offered to patients aged in each phase of CLL to offset any
>70 years. Clinical practice complications, particularly for
recommendations for the role of patients with high-risk features.
ASCT in the current management Supportive therapies given during
of relapsed CLL has relegated treatment for relapsed CLL may
its use to later in the disease. A include antibiotics, antivirals, or
recent paper summarising the antifungals to combat infection,
clinical findings of physicians and leukapheresis to separate out
with expertise of ASCT in CLL has large numbers of white blood cells
recommended ASCT for patients from the blood. For more details,
with the following: see the palliative care section
later in the booklet.
•• CLL which is refractory or
progresses despite treatment Clinical trials
with B-cell receptor inhibitors
For some patients, including
•• CLL in high-risk patients which those with poor treatment
responses associated with the
20 www.leukaemiacare.org.ukdel17p mutation, TP53 mutation response to treatment and your
and a lack of mutated IgVH, state of health. It is possible that
participation in clinical trials can your CLL will progress slowly and
offer another treatment option, remain controlled with targeted
as clinical trials are currently therapy. Alternatively, you may
making excellent progress in go into remission for many years,
the treatment of CLL. Moreover, during which time you will not
clinical trials represent a strong have any symptoms and your
contribution to CLL management, blood tests will be clear.
as well as access to newer
In the event of a relapse, you
medications which may not be
will be offered further treatment
available on the NHS, and the
to help you reach a second
possibility in the future with the
remission. If the new targeted
development of new treatments.
therapies are not successful,
Details of trials in the treatment you may be offered intensive
and management of CLL which are treatment with an ASCT,
recruiting patients can be found depending on your age and state
online at clinicaltrials.gov of health, but this is rare due to
the further treatments options
Prognosis that are now available.
The majority of patients will The two most important genetic
experience a relapse within prognostic markers for CLL that
five years of starting chemo significantly affect the course
immunotherapy, and for patients of the CLL and the likelihood of
with a duration of first remission relapse are:
less than three years prognosis is
worse. However, on the whole, CLL •• Chromosome 17 deletion 17p,
is not seen to have an impact on del(17p), which occurs in 30% of
natural lifespan. patients who relapse
Your prognosis will vary according •• TP53 mutations and/or no IgVH
to the phase of your CLL, your mutation
Helpline freephone 08088 010 444 21How is relapsed CLL treated?
(cont.)
The poor treatment responses,
and shorter time before relapse
associated with the del17p
mutation, TP53 mutation and lack
of mutated IgVH are the focus for
using new therapies to treat CLL.
22 www.leukaemiacare.org.ukHelpline freephone 08088 010 444 23
Seeing your doctor
Your symptoms Examples of questions to ask the
doctor:
Whatever symptoms you have,
make sure you write a list of all •• How would I know if I my CLL
of them to share with your doctor came back?
as they may be important to your
treatment.
•• What tests will I need to have?
•• What will the tests show?
Your appointment
Arranging an appointment with
•• How long will it take to get the
results back?
your general practitioner (GP)
or your consultant will be one •• How common is it to have a
of the first things you will need relapse?
to do when you start to notice
symptoms. Pick a time convenient •• What sort of treatment will I
for you that you know you will be need?
able to attend. If there is a chance •• How long will my treatment
that you might be experiencing last?
a relapse, you will be referred to
the hospital who will be able to •• How will I know if my treatment
confirm this through tests. has worked this time?
Your preparation •• What will the side effects be?
It is important to know exactly •• Are there any foods or
what you would like to ask medications I need to avoid?
your doctor. Make a list of your
questions and leave spaces for
•• Will I be able to go back to work?
the answers so you can write •• Where can I get help with
them down when you see the claiming benefits and grants?
doctor. This way you can go into
the meeting ready and prepared. •• Where can I get help dealing
with my feelings?
24 www.leukaemiacare.org.ukTalking to your doctor that nothing has been missed or
forgotten.
Be honest with your doctor; they
have seen and heard everything The next steps
before, so there is no need to feel
Always ensure that you leave the
embarrassed about anything. If
GP surgery, or the hospital, having
you saw your healthcare team
shared everything you know
before seeing your doctor, be
about your condition, with all of
sure to share with your doctor
your questions answered, and
everything your healthcare team
knowing exactly what the next
told you about your relapse, the
steps are, whether it is more tests,
blood tests you had performed,
further treatment or palliative
and the next steps in your CLL
care. You can ask for a summary
journey.
letter of the consultation to have
If you want to, ask if you will everything in writing. Your doctor
receive more intensive treatment will generally send a letter like
or palliative care. However, it is this to your GP.
important to remember that this
Furthermore, be sure to access
is only offered if other lines of
all of the other support available
treatment are exhausted or you
to you as this may be able to help
decide you no longer want further
you with your feelings towards
treatment. It is increasingly rare
your diagnosis and treatment.
for patients to die due to their CLL.
Your support
If it helps, take a family member
or friend in with you for support.
Some people take a pen and paper
in to make notes, and repeat back
to their doctor everything they
have been told to ensure that they
are both on the same page and
Helpline freephone 08088 010 444 25Telling your family
Planning who to tell people in an environment where
both of you can hear each other
Telling your family and friends clearly and where there are likely
that you have suffered a relapse to be no interruptions.
can be difficult, especially since
you already went through it with How to say it
your initial CLL diagnosis. One
Although it is upsetting that you
positive aspect to this is that you
have relapsed and the people
already have your team in place,
you tell may get upset for you, it
so once they know what your
is important to try and remain
situation is, they can help and
positive and optimistic when
support you like they did before.
talking. Even though a relapse
You may want to create a list of is unfortunate, it is not the end
people you want to tell, starting of the world, as there are further
with close family and friends, and treatment and supportive care
then extending it beyond, from options available.
your colleagues at work to friends
in your neighbourhood. How to respond
You may receive similar
Planning what to say responses to when you told people
It is important to know what about your initial diagnosis.
you want to say and exactly how Naturally they will feel sad, and
much you want people to know. concerned for you. Everyone deals
Being clear in your mind about with this type of news in their own
that before speaking to anyone way, from shock and silence, to
will make this a much smoother questions and support.
experience. Know your story that
Invariably, people respond
you want to tell, the diagnosis,
positively, which in turn means
the prognosis, the next treatment
you will respond back positively.
steps, and what you expect to
be going through physically and
emotionally. Be sure to speak to
26 www.leukaemiacare.org.ukAccepting help
You can receive help from
Sometimes people feel guilty for
their cancer relapsing, that they
us on how to speak to
weren’t strong enough, and that your friends and family
they will be a burden on those following a relapse.
around them. This is where your You can visit www.
loved ones come in, so make sure leukaemiacare.org.uk,
you do ask for and accept offers to or call 08088 010 444, to
help and support you in the next find out more.
stages of your CLL journey. Do not
try to cope on your own. If they
offer to help, tell them that you
will get in touch when you need
them.
Repeating yourself to different
people can become burdensome,
which is where your network of
family and friends can help you
out, by telling those beyond them
about your current situation.
Helpline freephone 08088 010 444 27Managing your emotions
Being told that your cancer has relapse and carrying on with your
returned may be difficult for life will help ease any anxieties.
you to deal with, especially after Help, care, kindness and support
all your time and effort during will be available to you from your
treatment and remission period. healthcare team, and you will
have access to counsellors and
Indeed, you may have a positive
therapists when you need it.
demeanour, which will obviously
be helpful to you during the next Isolation
steps in the management of your
CLL. However, you may experience If you have received a diagnosis of
a range of emotions, including relapse, and the next steps don’t
uncertainty, isolation, anxiety, involve intensive treatment, you
anger, sadness and depression. may feel a break in your routine.
Understanding each emotion and You may feel alone because
developing ways that help you you will no longer enjoy regular
deal with them will help you move meetings with your healthcare
forward with your life. team who provide you with
information and reassurance,
Uncertainty or fellow patients who can
provide you with empathy and
You may think "What happens
compassion.
next?". You may be unsure about
your health and what the future Alternatively, you may feel this
holds for you. You may or may break in routine allows you to
not have had meetings with your be around those closest to you,
healthcare team to discuss the and follow your regular routine of
next steps following your relapse. work and play. Being around those
Once you have a clear path set out closest to you, such as your family
in front of you, you will be able to and friends, can be positive and
develop a clearer picture of where negative.
you are headed. Gaining a sensible
Let them know what you do and
balance between being vigilant
don’t want to do, how you do
about your symptoms following
28 www.leukaemiacare.org.ukand don’t wish to be treated, help you to cope with the physical
and what you do and don’t feel effects of anxiety. Cognitive
comfortable talking about. behavioural therapy can help you
Sometimes, it is difficult for your deal with your worrying thoughts.
family, friends and colleagues
to understand what you are Anger
feeling and going through. Being Feeling angry after a relapse is
clear will help create the kind of natural and normal. You may be
positive, supportive, and caring angry with yourself, your body,
environment that will help as you with the healthcare team or
move forward with your life. with family and friends. You may
display your anger as impatience,
Anxiety irritability and frustration with
Being fearful of the unknown, people and things that would not
especially when we are feeling normally bother you.
threatened, is natural. You may
Understanding exactly what is
experience an increased heart
making you angry will help you
rate, rapid breathing, and muscle
deal with your feelings effectively.
tension. These things help us to
In addition, setting yourself
face a danger or run away. These
achievable goals that stretch you
changes in you are part of the
will help reduce the anger and
‘fight or flight’ response. Any
impatience you feel, especially
feeling of discomfort, pain or even
with each passing success. Don’t
another appointment with your
forget to congratulate yourself for
healthcare team may elicit such
each successfully completed task,
responses, and give you sleepless
however small.
nights or feelings of worry. This is
completely natural. Physical exercise is a great
way to release your anger and
Such reflexes and responses will
frustrations, and channel your
ease over time with the building
energy positively with no negative
of daily routines and planning
impact on your body. Talking
things for the future, which will
Helpline freephone 08088 010 444 29Managing your emotions
(cont.)
about your feelings, letting them do as much as you can and try
out, will also help stop you lashing and talk about your thoughts and
out at people and keep you calm. feelings. This will help lighten
your burden and put things into
Sadness and perspective. If you have made
depression any acquaintances or friends in
the same position as you, talk
Relapsing will bring back some,
to them over coffee as they will
if not all, of the feelings you felt
understand exactly what you are
when you were first diagnosed
facing.
with your CLL. You may feel a
sense of loss of the person you
Self-confidence
used to be, and how safe you felt.
You may also feel that your illness Being forced to readjust from your
is a heavy burden on those around daily routine during remission
you. You might be feeling low, back into one involving multiple
which is a natural effect of your visits to the hospital for further
illness, treatment and recovery. treatment, or moving into
However, if this low mood persists palliative care, can take its toll.
for more than several weeks, This interruption of your life, along
and you feel hopeless, and with your lack of energy because
lose interest and pleasure with of your CLL and the effects of your
things in life, then you may have treatment, can impact on how
depression. you feel about your appearance
and how you feel emotionally. In
Your first steps should be to turn, this can knock your self-
speak to your loved ones around confidence and self-esteem.
you about your mood and state Your feelings of relief, hope, and
of mind, and then contact your optimism have just been replaced
GP. You may lift the way you feel with their polar opposites.
by engaging in activities that
you were enjoying before your You can gradually build your self-
relapse and initial diagnosis, to confidence and self-esteem back
connect back with your life. Only up by engaging in the activities
30 www.leukaemiacare.org.ukyou did before your diagnosis, and can help quieten your mind and
socialising with family, friends, remove the stress of coming to
and fellow patients. This will help terms with your diagnosis, so you
create a supportive atmosphere to feel calmer and more relaxed.
get you back to your old self.
Mindfulness and
relaxation
Simple practices from
mindfulness and relaxation
techniques can help you calm the
mind, release tension and ease
any pain in your muscles.
•• Put yourself in a relaxing
environment, sitting or lying
down comfortably.
•• Loosen your clothing so you can
move more freely.
•• Calmly breathe in through your
nose, and out through your
mouth, developing a steady
natural rhythm, focusing on
your chest and abdomen as you
do so.
•• Visualise that you are inhaling
positivity and exhaling
negativity.
By taking some time out of your
day to do these exercises, you
Helpline freephone 08088 010 444 31Survivorship
Someone who is living with or is the end of treatment until the end
beyond a cancer diagnosis can be of life. At this point, your routine
considered a cancer survivor. of meeting frequently with your
healthcare professionals also
Survivorship can be defined as:
ends, so you may feel a mixture
"...cover[ing] the physical, of emotions from relief to fear,
psychosocial and economic anxiety and uncertainty about the
issues of cancer, from diagnosis future. You may wonder how you
until the end of life. It focuses will slot back into your life after
on the health and life of a coming through the treatment
person with cancer beyond the period.
diagnosis and treatment phases.
Your survivorship pathway began
Survivorship includes issues
at the point when you were
related to the ability to get health
diagnosed with CLL. By this point,
care and follow-up treatment, late
you will have been starting to
effects of treatment, secondary
receive support for work, finance,
cancers and quality of life. Family
and personal relationships
members, friends and caregivers
through to managing pain, fatigue
are also part of the survivorship
and making positive lifestyle
experience."
changes, such as starting a
When living with cancer, healthy diet and gentle exercising.
especially if you are relapsing
Your individual needs as a patient
after remission, you will face new
will be identified and addressed,
challenges to cope with from
including:
physical to psychological and
social ones. Survivorship aims •• Dealing with the emotional
to provide personalised care impact of receiving a diagnosis
based on your need to improve of relapse which may have
your health, wellbeing, quality created feelings of uncertainty,
of life, and your confidence and fears of recurrence and
motivation, to help you manage. difficulties in planning for the
Survivorship also focuses on your future. These will be discussed
health and life with cancer after with you to develop your
32 www.leukaemiacare.org.ukindividualised care plan with •• Preparing you fully for
support from social care staff the impact of relapse and
and therapists, as you need it. treatment, the physical and
physiological side effects
•• Improving your quality of life of treatments and the
through efficient and co-
psychological impact of CLL
ordinated care during your
relapse in general. You will be
treatment, with effective
provided physical equipment,
communication within the
and taught about various
treatment team, and a positive
coping strategies to adapt to
attitude.
your new situation.
•• Taking care of any comorbidities •• Supporting you with advice
– that is, other medical
for social and financial
conditions and diseases
difficulties, including caring
– and offering you cancer
responsibilities, your inability to
rehabilitation based on your
participate in social activities,
clinical needs as assessed by
any debt and financial worries
informed professionals, and
from not being able to work, and
ensuring compliance with the
perhaps the need to return to
National Cancer Rehabilitation
work before you feel ready.
Pathways.
•• Receiving health and nutrition
•• Providing you with a treatment advice from a nutritionist
summary from diagnosis of
on following a healthy and
your relapse to the end of your
balanced diet to help improve
treatment. This would include
your general health and
any ongoing medication and
wellbeing. The World Cancer
noting possible symptoms that
Research Fund published a
may occur in the future. You
report for cancer survivors
would also be provided details
which suggests that even small
of who to contact in addition to
dietary and lifestyle changes
your GP for any concerns you
can produce large health
may have.
benefits.
Helpline freephone 08088 010 444 33Palliative care
Palliative care in occupational therapists,
complementary therapists, and
relapsed CLL religious leaders, if you would like
Palliative care, also known as this. Your palliative care services
supportive care, involves a holistic may be provided by the NHS, local
or "whole person" approach, which council or a charity. You may
includes the management of your receive day-to-day care at your
pain and symptoms as well as home and at the hospital.
psychological, social and spiritual
support for you and your loved What is the clinical
ones. course?
Palliative care aims to reduce You will have experienced a
your symptoms, control your CLL, protracted cycle of relapses and
extend your survival, and give remissions, and be prone to
you and your loved ones the best frequent infections because of
quality of life possible. Your doctor the CLL and the impact of your
will discuss the options with you treatments. Your chemotherapy
in detail before you decide the may continue because of
next steps. potential remission and/or useful
palliation.
Who provides palliative
You may experience various pains
care? and other clinical complications
Your palliative care will be such as:
provided by a team of health and
social care professionals trained ••Bone pain: Radiotherapy and/
in palliative medicine who will or oral steroids, and sometimes
coordinate your care. non-steroidal anti inflammatory
drugs (NSAIDs), may be used,
These professionals can include although these are used with
your GP, hospital doctors and caution because they can
nurses, community nurses, interfere with your immune
hospice staff and counsellors, system and kidney function.
social care staff, physiotherapists,
••Bone marrow failure: Blood
34 www.leukaemiacare.org.ukand platelet transfusions vertebrae of the spinal column:
are provided to prevent and Treatments can include
fight recurrent infections and analgesics, antidepressants
bleeding episodes. and/or anticonvulsant
medication used in tandem
••Oral problems: Analgesic with opioids.
mouth washes and topical
ointments may help with ••Hypercalcaemia: Treatment
ulceration. Chewing gum, and is usually with intravenous
mouth washes have been shown hydration and intravenous
to help with dry mouth, dental bisphosphonates.
caries and oral thrush.
••Loss of appetite: Low-dose
••Night sweats and fever: These steroids may temporarily
can place a heavy burden on boost the appetite, while small,
carers because of so many frequent and appetising meals
changes of night clothes and and supplement drinks will also
bedding. help.
••Pathological fractures:
Orthopaedic intervention and
subsequent radiotherapy,
with consideration given to
prophylactic pinning of long
bones and/or radiotherapy
to prevent fractures will be
performed. This will reduce
the likelihood of complex pain
syndromes developing.
••Spinal cord compression:
Immediate high single daily
dose oral steroids will be given.
••Back pain from wedge
and crush fractures of the
Helpline freephone 08088 010 444 35End of life care
When does end of life Who provides end of
care begin? life care?
If you have relapsed and are going A team of health and social care
through palliative care, you may professionals may be involved
be offered end of life care. End of in your end of life care, including
life care begins when you need it hospital doctors and nurses, your
and may last a few days, months GP, community nurses, hospice
or years. staff and counsellors, social
care staff, physiotherapists,
What does end of life occupational therapists or
care involve? complementary therapists, and
religious leaders, if you would like
End of life care is support for
this. If you are being cared for at
people who are in the last few
home or in a care home, your GP
months or years of their life. The
will have overall responsibility for
aim is to help you enjoy a good
your care with the support from
quality of life until you die, and to
community nurses, along with
die with dignity. The professionals
your family and friends.
looking after you will ask
you about your wishes and
What choices do I have
preferences on how to be cared
for and put these into action. They in terms of end of life
will also provide support to your care?
family, carers and loved ones. You Deciding where you want to die
will be able to decide where you can be a difficult choice to make.
will receive end of life care, be it at Working out what you and your
home or in a care home, hospice loved ones want, together with
or hospital. The same will be true seeing what services are available
of where you would like to die. to you can help to make the
Wherever you are, you will receive decision a little easier.
high quality end of life care.
••Staying at home - A place of
familiarity, surrounded by your
loved ones, may be something
36 www.leukaemiacare.org.ukthat you will find reassuring. cannot always be tailored to
External care professionals will your specific needs. Pressures
be able to visit you at home to on the NHS mean that your
make sure your symptoms are stay will only be as long as
looked after. strictly required. As soon as the
condition you were admitted
••Hospices – Specialised in for has been resolved, you will
looking after those with life-
need to go back to your home
limiting illnesses and those
or nursing home. However, a
who are coming to the end of
number of specialists will be
their life, hospices are staffed
available to help look after you
with care professionals who
for specific problems, and a
are able to keep an eye on you,
number of hospitals also have a
make sure that your symptoms
designated palliative care team
are controlled and offer you a
for patients who require them.
number of services to make
your stay as comfortable as Whatever your choice, speak
possible. For more information with your GP or healthcare team
on the care that they can who will able to help you put
provide, go to https://www. everything into place.
hospiceuk.org/
••Residential care/nursing
homes - If you think that your
stay may be a few months or
more, then a nursing home may
be more suitable than a hospice.
These can be private or run by
a charity or the local council so
be sure to check if there are any
fees.
••Hospitals - Although you
may be used to staying in a
hospital ward, the care routine
Helpline freephone 08088 010 444 37Glossary
Allogeneic Stem Cell Transplant cells are not fully developed and
(ASCT) are called blasts or leukaemia
cells.
Stem cell transplant of cells from
a matching donor. Bone Marrow Failure
Amino Acids Term used when the bone marrow
is unable keep up with the body’s
Organic molecules which are
need for white and red blood cells
the building blocks for making
and platelets.
proteins.
Central Nervous System
Anaemia
Part of the nervous system which
Condition where the number of
includes the brain and spinal
red blood cells, which contain
cord.
haemoglobin and transport
oxygen to body cells, are reduced. Chemotherapy
This may be due to a lack of iron,
Drugs that work in different ways
leukaemia or sickle cell disease.
to stop the growth of cancer cells,
Antibody either by killing the cells or by
stopping them from dividing.
Protein produced by the B-cell
lymphocytes in response to Chromosomes
a specific antigen, such as
Thread-like structures which carry
a bacteria, virus, or foreign
the genes, and are located in the
substance in the blood.
nuclei of every cell in the body.
Antigen There are 46 chromosomes (23
pairs) in humans.
Toxin or other foreign substance
which induces an immune ClinicalTrials.gov
response in the body, especially
ClinicalTrials.gov is a database
the production of antibodies.
of trials and includes details of
Blasts 276,190 research studies in 204
countries.
Patients with leukaemia have a
high number of abnormal white
blood cells. These white blood
38 www.leukaemiacare.org.ukClonal found in the nucleus of each cell
in the body which carries genetic
Refers to an organism descended instructions used in the growth,
from, and genetically identical, to development and functioning of
a single common ancestor. the individual’s cells.
Complete remission Fatigue
Complete remission is said to Tiredness and weakness
have occurred when the following rendering the patient unable to
conditions have been met: work or perform usual activities.
•• Blood cell counts returned to Flow Cytometry
normal
Technology used to analyse
•• Less than 5% of blasts the physical and chemical
(abnormal, immature, early characteristics of particles in a
lymphocytes) are still present in fluid as it passes through at least
the bone marrow one laser. Cell components are
fluorescently labelled and then
•• There is no leukaemia present excited by the laser to emit light
elsewhere in the body
at varying wavelengths.
Cytogenetics
Fluorescence in Situ
Branch of genetics that is Hybridisation (FISH)
concerned with how the
Process using fluorescent
chromosomes relate to cell
dyes to attach to certain parts
behaviour, particularly to their
of chromosomes for their
behaviour during division and
identification.
reproduction.
Cytotoxic Drugs Genes
Genes are made up of DNA which
Drugs that are toxic to cancer
stores the genetic information
cells and prevent their growth and
required to make human proteins.
replication.
DNA (Deoxyribonucleic Acid) Immunophenotyping
Process that uses antibodies to
Thread-like chain of amino acids
Helpline freephone 08088 010 444 39Glossary (cont.)
identify cells based on the types system (part of the body’s
of antigens or markers on the immune system) that contain
surface of the cells. This process lymphocytes which produce
is used to diagnose specific types antibodies and macrophages to
of leukaemia and lymphoma digest dead cells. Lymph nodes
by comparing the cancer cells are swollen with cell fragments in
to normal cells of the immune the event of infection or cancer.
system. They are located mainly in the
spleen but also in the neck,
Immunotherapy armpit and groin.
Treatment that uses the body’s
own immune system to fight the Minimal Residual Disease
cancer. (MRD)
Measure of the presence of
Leukopenia leukaemia at a molecular level
Abnormally low number of white rather than at a cell level. It
blood cells in the blood. is measured using molecular
techniques such as flow
Leukaemia cytometry and polymerase chain
A group of cancers that usually reaction analysis.
begin in the bone marrow
and result in high numbers of Morbidity
abnormal white blood cells. These Refers to having a disease or a
white blood cells are not fully symptom of a disease.
developed and are called blasts
or leukaemia cells. Depending Phase 1 Trial
on the type of white blood cell Small trial (up to 30 volunteers)
involved, there are different to confirm if the drug behaves
types of leukaemia with varying as expected, determine its side
characteristics, such as being effects, and how the body reacts
acute (develop quickly) or chronic to the drug.
(develop slowly).
Phase 2 Trial
Lymph Nodes Medium trial (up to 100
Components of the lymphatic volunteers/patients) to confirm
40 www.leukaemiacare.org.ukthe drug’s safety, find the best Protein Kinase Inhibitor
dose to use, assess the drug’s
Protein kinase inhibitors block
effectiveness if the trial is in
the protein kinase enzymes that
patients, and determine the value
are involved with cell growth,
of studying it in large numbers of
thereby preventing the growth of
patients in a Phase 3 trial.
the cancer cells.
Phase 3 Trial
Refractory
Large clinical trial (more than
Refractory CLL occurs when the
100 patients) that collects
cancer has not responded to first-
information on a drug’s safety
line treatment.
and effectiveness using different
populations and different Relapse
dosages, and by comparing it to
A relapse is when a patient
other known drugs for a condition.
initially responds to leukaemia
Phase 4 Trial therapy but, after six months or
more, response stops. This is also
Trial conducted once a drug has
sometimes called a recurrence.
been granted a licence to find out
more about a drug’s side effects, Remission
its long-term risks and benefits,
Remission occurs when the
or how well it works when it’s used
following conditions are met:
more widely.
Platelets
•• Blood cell counts returned to
normal
One of the types of blood cell
which helps to stop bleeding. •• Less than 5% of blasts (early
stem cells) are still present in
Prognosis the bone marrow
Indication of how well a patient is •• There is no leukaemia present
expected to respond to treatment elsewhere in the body (minimal
based on their individual residual disease)
characteristics at the time of
diagnosis or other timepoint in Salvage Chemotherapy
the disease. Chemotherapy given to a patient
Helpline freephone 08088 010 444 41You can also read
