Splenic marginal zone lymphoma Freephone helpline 0808 808 5555 www.lymphomas.org.uk

Freephone helpline 0808 808 5555
information@lymphomas.org.uk
www.lymphomas.org.uk
Splenic marginal zone lymphoma
Splenic marginal zone lymphoma (splenic MZL) is a slow-growing type of non-Hodgkin
lymphoma. Non-Hodgkin lymphoma is a cancer of the lymphatic system, which is part
of our immune system. Splenic MZL develops in the spleen, an organ in the upper
abdomen. This lymphoma usually affects people over the age of 50, but it can occur in
younger people too.
In this information sheet we aim to answer the questions people ask about this
lymphoma:
• What is splenic MZL? (see below)
• What causes splenic MZL? (page 3)
• What are the symptoms of splenic MZL? (page 3)
• What tests will I need? (page 4)
• How is splenic MZL treated? (page 6)
• What happens if a splenic MZL comes back? (page 7)
We have separate information on other types of marginal zone lymphoma. If you
would like information on nodal MZL or MALT lymphoma, please ring our helpline
on 0808 808 5555 and they can send you this. Alternatively, you can download
the information from our website (www.lymphomas.org.uk).
What is splenic MZL?
What is lymphoma?
A lymphoma is a cancer of cells called lymphocytes, which are white blood cells
that normally help the body to fight infections. There are two kinds of lymphocyte,
B lymphocytes (usually just known as 'B cells') and T lymphocytes (T cells).
B cells are made in the bone marrow, which is a spongy tissue found in the middle of
some of our bigger bones. T cells start their life in the bone marrow but they mature in
the thymus, a gland in the chest which lies just behind the sternum or breastbone.
Lymphocytes collect in lymph nodes (glands). Lymph nodes are found in groups,
particularly under the arms, in the neck and in the groin. We also have groups of lymph
nodes internally, around our organs. Lymphocytes can also be found in other parts
of the body, such as in the spleen and the thymus. These places are all part of our
lymphatic system (see Figure 1 on page 2).
If lymphocytes start to divide and multiply uncontrollably or if they don’t die off after
their normal lifespan, they can build up and form a lymphoma.
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Figure 1: The lymphatic system
Neck (cervical)
lymph nodes
Lymph vessels
Thymus
Diaphragm
(muscle that
separates the
chest from
the abdomen)
Armpit (axillary)
lymph nodes
Spleen
Liver
Groin (inguinal)
lymph nodes
What types of lymphoma are there?
Lymphomas can develop from either type of lymphocyte. There are therefore B-cell
lymphomas and T-cell lymphomas. If a lymphoma develops and grows quickly it is
called a ‘high-grade’ or ‘aggressive’ lymphoma. If it is slow-growing it is a ‘low-grade’
or ‘indolent’ lymphoma.
There are over 60 types of lymphoma, but there are two main kinds – Hodgkin
lymphoma and non-Hodgkin lymphoma. These look different when they are examined
under the microscope in the laboratory. They also affect people differently.
What kind of lymphoma is splenic MZL?
Splenic MZL is a low-grade B-cell non-Hodgkin lymphoma that develops in the spleen.
The lymphoma is usually also present in the bone marrow and is often found in the
blood too. It can affect the internal lymph nodes next to the spleen and it can affect
the liver. Less than 2% of non-Hodgkin lymphomas will be of this type, so it is a rare
lymphoma.
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Splenic MZL is described as a ‘marginal zone lymphoma’ because the lymphoma cells
were originally thought to be cancerous forms of B cells from a part of the spleen
called the marginal zone. Exactly how splenic MZL develops is controversial, however,
and it might be that this lymphoma will be renamed one day when more is known
about which cells it develops from.
What is the spleen?
The spleen is an organ in the upper part of the abdomen, on the left-hand side. It
is normally about the size of a clenched fist. It has two main jobs to do. Firstly, it
is part of the body’s immune system and it helps us to fight infections. Its second
main function is to filter out and remove old and damaged blood cells from the
bloodstream.
What causes splenic MZL?
Lymphocytes start to grow out of control and form a lymphoma because something
happens to change or damage their genes. If their genetic make-up changes they
can lose their ability to control how quickly they divide or die off. It is not known why
these genetic changes happen in the lymphocytes in most lymphomas.
Some people with splenic MZL show signs of past or current infection with the
hepatitis C virus. This type of splenic MZL is more common in some parts of southern
Europe, such as Italy. In this type of splenic MZL, tests show abnormal lymphoma
cells in the circulation. These cells are often seen to have irregular villous (finger-like)
projections on their surface when they are examined under the microscope. These
are therefore called 'villous lymphocytes' and this type of splenic MZL is sometimes
called 'splenic lymphoma with villous lymphocytes' or SLVL.
Most people with splenic MZL will show no sign of infection, however – and not
everyone who has hepatitis C develops a splenic lymphoma. This means that there
must be other reasons for the lymphocytes losing control and forming a splenic MZL.
Researchers are trying to find out more about why these lymphomas develop in some
people who have had a hepatitis C virus infection but not in others. It is important that
researchers find out as much as possible about splenic MZLs and how they develop,
so that more targeted and effective treatments can be developed.
What is known is that nothing you have done – or not done – will have caused the
lymphoma to develop. Also, splenic MZL is not a disease that you can inherit or pass
on to your children.
What are the symptoms of splenic MZL?
Many people will have no symptoms at all when splenic MZL is first diagnosed. It is
sometimes discovered during an examination of your abdomen or when blood tests
are done for some other reason.
If the spleen becomes enlarged by the lymphoma – this is called splenomegaly – this
can cause discomfort or pain in the upper part of the abdomen.
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Some people with splenic MZL will have low blood cell counts. This happens mainly
because the enlarged spleen is filtering out too many blood cells. It can also happen if
there is lymphoma taking up space in the bone marrow. When there is lymphoma in
the bone marrow it is not able to manufacture as many blood cells as it did before.
The low blood counts can make you:
•tired, or short of breath when you exercise – due to a shortage of red blood cells
(anaemia)
•prone to bruise or bleed easily – due to a shortage of platelets, which are cell
fragments that help the blood to clot (this is called thrombocytopenia).
Sometimes the lymphoma cells produce a small amount of an abnormal protein called
a ‘globulin’ and this is found in the bloodstream. This protein can make the blood
thicker and more slow-moving than normal. This can cause headaches, confusion,
nosebleeds and blurred vision.
A few people experience more general symptoms such as weight loss, fevers and
night sweats but these are not common. Unlike most other lymphomas it is not
common to develop lumps due to enlarged lymph nodes in splenic MZL.
What tests will I need?
You will have a physical examination and lots of tests. It is important to make sure that
your symptoms aren’t being caused by another condition, for example by an infection
or a blood disease. This means that some tests will be done to rule these other
possibilities out.
You will have tests done to find out exactly what kind of lymphoma you have – to
diagnose the lymphoma. The medical team will need to know this before they can
plan your treatment. Some tests will be done to find out how much of the body is
affected by the lymphoma. You might hear these tests referred to as 'staging' tests.
Don’t worry if you don’t seem to be having the same tests as other people you meet
who have lymphoma. The medical team will order all the tests that are necessary for
your situation, depending on your symptoms and what they find when they take your
history and examine you.
You are likely to have:
• blood tests:
– to check for anaemia or other low blood cell counts
– to see if there are any villous lymphocytes in the blood
– to check the levels of several chemicals in the blood and to see if your kidneys
and liver are working well
– to see if you have any signs of a hepatitis C virus infection.
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• scans:
– CT scan (computed tomography) – this is the most usual scan people have. This
kind of scan produces pictures of your internal organs in cross-section, from top
to bottom, and will show up internal organs such as the spleen and liver as well as
internal lymph nodes
–u
ltrasound scan – this is used in some centres to examine the spleen. This is a
scan that uses sound waves to show up tissues and organs
– PET scan (positron-emission tomography) – this kind of scan is sometimes done if
the specialist feels it would be helpful in planning your treatment.
•
bone marrow biopsy – this test involves taking a sample of bone marrow through
a needle. The needle is usually inserted through your skin into your hip bone after
a small area of skin has been numbed using a local anaesthetic. A bone marrow
biopsy takes around 15 minutes.
In addition to these tests, if you have your spleen removed (see page 6) this will be
sent to the laboratory to be examined.
The samples from the bone marrow, the blood (and the spleen if this was removed)
will be sent to the laboratory. They will be examined under the microscope. Detailed
tests will be done to find out the exact type of lymphoma you have.
The doctor who examines the samples (the histopathologist) will look at the types of
proteins on the surface of the lymphoma cells (immunohistochemistry tests). They
may also examine the genetic make-up of the lymphoma cells (cytogenetics tests). The
results of these specialised tests will tell the medical team which kind of lymphoma is
affecting the spleen. They can take 2–3 weeks to come back.
This information sheet is about splenic MZL, which is the most common lymphoma
that develops in the spleen. Other types of non-Hodgkin lymphoma that can be found
in the spleen are mantle cell lymphoma, follicular lymphoma, lymphoplasmacytic
lymphoma, Waldenström’s macroglobulinaemia, hairy cell leukaemia, hairy cell
leukaemia-variant, splenic diffuse red pulp small B-cell lymphoma and diffuse large
B-cell lymphoma. It is important to find out exactly which type you have because these
lymphomas behave differently and need to be treated differently.
Because so many lymphomas can affect the spleen, splenic MZL can be tricky to
diagnose. This means that you might have to wait for some time before test results
come back from specialist laboratories and sometimes tests have to be repeated. It is
difficult to wait for results when you are feeling anxious, but it is important to have an
accurate diagnosis so that you can be started on the most suitable treatment for your
kind of lymphoma.
Please ring the helpline on 0808 808 5555 if you would like to talk to someone about
your lymphoma or if you would like information on tests on or on other types of
lymphoma sent to you. You can also download information about lymphoma from our
website (www.lymphomas.org.uk).
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How is splenic MZL treated?
Like other types of low-grade non-Hodgkin lymphoma, splenic MZL is rarely
considered to be curable, but there are very effective treatments available. Doctors
will be aiming to get you into a long-lasting remission (when tests will show no signs
of active lymphoma).
The treatment will depend on your symptoms and your blood counts. If your spleen
isn’t large enough to be causing discomfort and you don’t have low blood counts it is
common to have no treatment at all for a while. You will be checked regularly in the
outpatient clinic. This period of regular observation is called watch and wait.
This might seem unexpected when you have been diagnosed with a type of cancer,
but it has been shown that having early treatment does not offer any advantages in
this situation. Many people with this lymphoma have been able to delay having any
treatment for 10 years or more in this way.
If the enlargement of the spleen is causing symptoms such as pain or if your blood
counts are low, the medical team will begin to treat the lymphoma.
Possible treatments are:
•
surgery – removal of the spleen, an operation called splenectomy. If the spleen
hasn't already been removed in order to diagnose your lymphoma, it might be
removed if the spleen becomes very uncomfortable or if the blood counts are
very low. This operation can be done using keyhole surgery if the spleen isn't too
enlarged. You can live without a spleen but there are precautions that you need to
take after having a splenectomy (see page 7).
•antibody treatment – treatment with a specially manufactured protein antibody
such as rituximab. Rituximab is used more commonly than surgery nowadays
and is a very successful treatment in many people. It is sometimes given with
chemotherapy.
•
chemotherapy – treatment with just one chemotherapy drug, such as chlorambucil
(which is taken as tablets), cyclophosphamide, fludarabine or cladribine; or with a
combination of several chemotherapy drugs. Rituximab is sometimes given with
the chemotherapy.
•
splenic irradiation – treatment with low doses of radiotherapy to the spleen. This
kind of treatment might be suggested if the enlarged spleen is causing symptoms
but the medical team feel that you are not well enough to have surgery.
• antiviral treatment (with interferon and ribavirin) – this will be given if the blood
tests show signs of a hepatitis C virus infection.
Research is being done to find other possible treatments for splenic MZL. You might
be offered an opportunity to have one of these newer drugs as part of a clinical trial.
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Follow-up after your treatment
Once the treatment is finished you will be seen in the clinic every 1–3 months at first,
then every 6 months after a year or so for a further 2 years. Eventually you will be
seen once a year. How long you will be followed up for will depend on the treatments
you had and this also varies from centre to centre. If the specialist feels they would be
helpful, you might have blood tests and scans done during your follow-up, but they are
not done routinely.
Precautions to take after having a splenectomy
You can live quite normally without a spleen, but there are certain precautions you
will need to take from now on because you will be more susceptible to complications
if you get infections. You will be given detailed information about the operation and
about how to look after yourself afterwards.
You will need to have some vaccinations before your operation. It is important that
you have your pneumococcal and flu vaccinations regularly afterwards too. It is
recommended that you have the flu jab every year and the pneumococcal jab every
3–5 years.
You might be given small doses of antibiotics to take every day after your operation.
If you feel unwell, for example with a sore throat, sore stomach or headache,
consult your doctor early in case an infection is starting. You will have to take special
precautions if you are ever bitten by a dog or if you plan to travel to areas where there
is malaria.
You should always tell any doctor or dentist you see that you have had your spleen
removed and it is advisable to wear a MedicAlert® bracelet or to carry a warning card
about with you.
Further information on splenectomy is available at:
•the Patient.co.uk website, which has good information for people who have had a
splenectomy (www.patient.co.uk/health/preventing-infection-after-splenectomy-or-ifyou-do-not-have-a-working-spleen)
•the NHS Choices website, which has a section called 'How can I prevent infection
if I don't have a working spleen'. This includes links to and information about
splenectomy warning cards and MedicAlert® bracelets (see: http://www.nhs.uk/chq/
pages/2390.aspx).
What happens if splenic MZL comes back?
Splenic MZL usually responds well to treatment. After having a splenectomy, for
example, the lymphoma commonly needs no further treatment for many years (the
average time is 8 years). It can relapse (come back) however. This would be very
disappointing, but relapsed splenic MZL can be successfully treated again. Most
people with relapsed splenic MZL will be offered another course of chemotherapy
and/or antibody treatment.
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Rarely, people with splenic MZL will go on to need more intensive treatment because
their lymphoma has changed into a faster-growing, high-grade type of lymphoma.
This is called 'transformation'. If this happens the lymphoma would be treated in the
same way as a high-grade non-Hodgkin lymphoma, with a more intensive course of
treatment. A combination of rituximab and several chemotherapy drugs is often given
in this situation, for example R-CHOP (rituximab combined with cyclophosphamide,
hydroxydaunorubicin, vincristine [Oncovin®] and prednisolone).
We have separate information on watch and wait, chemotherapy, antibody
treatments and splenectomy. We also have information on transformation and
a booklet about taking part in clinical trials. Please ring the helpline if you would
like to talk to someone about your treatment or if you would like any of this
information sent to you (0808 808 5555). The information is also available on our
website (www.lymphomas.org.uk).
Acknowledgement
We are grateful to Dr Andrew Wotherspoon, Consultant Histopathologist at The Royal
Marsden Hospital, London for reviewing this information.
Useful organisations
Macmillan Cancer Support
0808 808 0000 (Monday–Friday, 9am–8pm)
www.macmillan.org.uk
CancerHelp UK
The patient information section of Cancer Research UK.

0808 800 4040 (Monday–Friday, 9am–5pm)
www.cancerresearchuk.org
Sources used in this information
Our information is written using the most up-to-date published research available
and current nationally recognised guidelines. If you want to know which textbooks,
guidelines and research papers we used as sources for this information, please
contact us by emailing the publications team on publications@lymphomas.org.uk or
ring us on 01296 619409.
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How we can help you
We provide:
a Freephone helpline providing information and emotional support  0808 808 5555
(9am–6pm Mondays–Thursdays; 9am–5pm Fridays) or  information@lymphomas.org.uk
●
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booklets and other information about lymphoma (free of charge)
●
a website with forums – www.lymphomas.org.uk
●
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the opportunity to be put in touch with others affected by lymphoma through our
buddy scheme
a nationwide network of lymphoma support groups.
How you can help us
We continually strive to improve our information resources for people affected by
lymphoma and we would be interested in any feedback you might have. Please visit
www.lymphomas.org.uk/feedback or email publications@lymphomas.org.uk if you
have any comments. Alternatively please phone our helpline on 0808 808 5555.
We make every effort to ensure that the information we provide is accurate but it should
not be relied upon to reflect the current state of medical research, which is constantly
changing. If you are concerned about your health, you should consult your doctor.
The Lymphoma Association cannot accept liability for any loss or damage resulting from
any inaccuracy in this information or third party information such as information on
websites which we link to. Please see our website (www.lymphomas.org.uk) for more
information about how we produce our information.
© Lymphoma Association
PO Box 386, Aylesbury, Bucks, HP20 2GA
Registered charity no. 1068395
Produced: January 2014
Next planned review: 2016
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