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Burkitt lymphoma
Burkitt lymphoma is a cancer of the lymphatic system, a type of non-Hodgkin lymphoma.
Even though more than 12,000 people are diagnosed with non-Hodgkin lymphoma in the
UK every year, many people have never heard of it before. Even fewer people have heard of
Burkitt lymphoma.
This leaflet aims to answer the main questions that people diagnosed with Burkitt lymphoma
or their relatives might ask:
What is Burkitt lymphoma?
What causes Burkitt lymphoma
and who is affected by it?
Figure 1: The lymphatic system
What are the symptoms of Burkitt
How is it diagnosed and what
further tests might I need?
What is meant by ‘the stage’?
How is Burkitt lymphoma treated?
What happens after my initial
Neck (cervical)
lymph nodes
Lymph vessels
Armpit (axillary)
lymph nodes
What is lymphoma?
Groin (inguinal)
lymph nodes
(muscle that
separates the
chest from
the abdomen)
Lymphoma is a cancer that develops
when a type of white blood cell called
a lymphocyte grows out of control.
Lymphocytes usually fight infections
and often collect in our lymph nodes
(glands). These lymph nodes are part
of our lymphatic system, which forms
part of our immune system. Lymphatic
tissue also occurs in other places such
as our lungs and gut. In these areas,
lymphocytes may be needed to fight infections that we breathe in or swallow with our food.
Lymph nodes are found in many areas of our bodies: some are easily felt if they are enlarged;
others are internal and may only be seen on scans. Our lymph nodes act like sieves in
our lymphatic system, and lymphocytes often collect in them. If our bodies make more
lymphocytes, for example to fight a sore throat, our nodes may swell until the infection
has gone.
Burkitt lymphoma
In the same way, a build-up of cancerous lymphocytes can make lymph nodes swell. They may
collect in other places too, such as the spleen, liver, gut, skin and bone marrow. When these
organs are involved by lymphoma, they are called ‘extranodal sites’.
There are many different types of non-Hodgkin lymphoma. These can be divided by the type
of lymphocyte that has turned cancerous. So B-cell lymphomas arise in cells known as
B lymphocytes; T-cell lymphomas in T lymphocytes. They can also be divided into high-grade
(or fast-growing) lymphoma and low-grade (or slow-growing) lymphoma.
What is Burkitt lymphoma?
Burkitt lymphoma is named after a doctor called Denis Burkitt, who first wrote about this
kind of tumour in children and young adults in Africa. The type of Burkitt lymphoma he
described is called the ‘African-type’ (or ‘endemic’) Burkitt lymphoma. Another type of
Burkitt lymphoma (the ‘sporadic’ type) is more often seen in the Western world and can
affect anyone. This leaflet contains information mainly about the sporadic type.
Burkitt lymphoma is quite rare: only 1 in every 30–50 people with a B-cell non-Hodgkin
lymphoma will have this type of lymphoma. It is one of the fastest growing lymphomas, but this
actually helps when it comes to treatment. It means that it can often be cured with intensive
What causes Burkitt lymphoma?
In some cases, Burkitt lymphoma is linked to previous infection with the Epstein–Barr virus
(EBV), the virus that causes glandular fever. For the endemic type, this is almost always the
case. EBV is a common virus: 9 out of 10 adults in the UK have evidence in their blood of
previous infection. It is not clear why some people develop lymphoma whilst most do not.
The virus seems to cause prolonged stimulation of some people’s normal B cells. This
increases the chances of the cells becoming cancerous, but is not the whole story.
Although this lymphoma is sometimes linked to a virus, it is important that you know:
You cannot catch lymphoma.
You did not inherit it from your parents.
You cannot pass it on to others.
Who is affected by Burkitt lymphoma?
Burkitt lymphoma can affect both adults and children. It makes up over a third of all the
lymphomas seen in children and is relatively common in adolescents and young adults. It is
now also being recognised more often in those aged over 60. The endemic type of Burkitt
lymphoma is typically seen in children, mainly in central Africa.
Over two-thirds of the adults with Burkitt lymphoma are men. In children, boys are more
likely to be affected than girls.
People who have HIV/AIDS (human immunodeficiency virus/ acquired immune deficiency
syndrome) are more likely to develop Burkitt lymphoma. This is probably because HIV, like
EBV, may over-stimulate the B cells. Many patients with Burkitt lymphoma will not have HIV or
Burkitt lymphoma
AIDS. However, your doctors will almost certainly recommend you have a test to rule out HIV
infection. This is because HIV is now very treatable and it is important that everyone with HIV
and Burkitt lymphoma receives effective therapy for both conditions. The outcome for people
with Burkitt lymphoma who also have HIV has improved rapidly in the last few years.
What are the symptoms of Burkitt lymphoma?
The symptoms of Burkitt lymphoma often come on over just a few weeks because the
lymphoma cells are dividing so quickly. For the same reason, it is common for the lymphoma
to be in several places by the time it is diagnosed.
You will probably have swelling of one or more lymph nodes in different parts of
your body. Many people with Burkitt lymphoma have lymphoma affecting their bowels. If your
bowels are affected, you may have abdominal pain, sickness and/or diarrhoea. These
symptoms may sometimes even be confused with appendicitis. The lymphoma might also
cause a collection of fluid within your abdomen (called ‘ascites’) or might cause your bowel to
become obstructed or bleed.
Burkitt lymphoma is often found in extranodal sites, so you are likely to have other areas
involved too. You may also have other symptoms of lymphoma, including night sweats,
tiredness, flu-like symptoms and unexplained weight loss.
Many people have Burkitt lymphoma in their bone marrow. The presence of lots of cancerous
cells in the bone marrow can stop it making healthy blood cells. You may develop anaemia
(due to a shortage of red blood cells). Anaemia causes shortness of breath, bone pain and
fatigue. Or you may have thrombocytopenia (a shortage of platelets). Thrombocytopenia
means you are more likely to bleed and that your body will be less able to stop any bleeding.
The lymphoma cells can sometimes be found in the bloodstream too, which is similar to
It is not unusual for Burkitt lymphoma to involve the brain and spinal cord. This is known as
your central nervous system (CNS). If affected, your symptoms will vary: you may have
no symptoms or you could have headache, seizures, confusion or inability to concentrate.
Burkitt lymphoma may be found in other organs such as the spleen, liver, kidneys, ovaries and
breasts. In children with the endemic type, Burkitt lymphoma often affects the jaw. It seems to
grow in the areas where the permanent teeth are forming. This rarely happens in the sporadic
(Western) type.
How is Burkitt lymphoma diagnosed?
Burkitt lymphoma is usually diagnosed by biopsy of an enlarged lymph node or another
affected tissue. You may already have had your biopsy, as generally no one will be able to say
for sure that you have Burkitt lymphoma until after this is done. The biopsy is examined by a
team of laboratory specialists, including a specialist pathologist, using a variety of tests. Better
and more advanced tests for this condition have been found in recent years and studies
continue to work out the best ones to do. This is because it is very important to correctly
identify Burkitt lymphoma. It needs to be treated differently from most high-grade lymphomas.
Burkitt lymphoma
The tests include looking at the cells under a microscope and looking for specific proteins on
and within the cells and for changes within the cells’ genes. In Burkitt lymphoma, these tests
will show that almost all of the cells are actively dividing. They will also show that one gene,
known as myc, has changed position and is overworking. It is this that makes the cells divide so
Some of these tests may take a while to complete so it may be a few weeks before your exact
diagnosis is certain. If there is any doubt, you will probably be started on therapy for Burkitt
lymphoma while the results are awaited as treatment should not be delayed. If it turns out that
you have another type of high-grade lymphoma, your doctors will then make any changes that
are needed to your treatment.
What further tests may be performed?
Other tests that might be done before you start treatment for your lymphoma may include:
Blood tests
- to check for anaemia or other low blood counts
- to check the salts in your blood and how your kidneys and liver are working
- for LDH (lactate dehydrogenase), which measures your lymphoma’s activity
- for infections such as HIV and hepatitis, which can flare up with lymphoma treatments.
Bone marrow test. This involves a sample taken through a needle inserted into a bone
at the back of your pelvis (above your hip). The area is numbed with a local anaesthetic but
you may still feel some discomfort.
Scans, which may include:
-Computed tomography (CT) scan, which uses X-rays to produce an image of your
internal organs and lymph nodes.
-Magnetic resonance imaging (MRI) scan, which may provide better images of certain
parts of the body such as the brain and nerves.
-Positron-emission tomography (PET) scan, which is a relatively new test, is proving to
be useful. It will not be done for everyone with Burkitt lymphoma as it might not be
available quickly enough or within your hospital. It is much more important to start
treatment promptly.
Lumbar puncture. This removes a small sample of the fluid that cushions your spinal
cord and brain (known as ‘cerebrospinal fluid’). The sample is taken using a needle inserted
between the spinal bones in your lower back after the skin has been numbed with a local
anaesthetic (see Figure 2).
Cardiac tests such as electrocardiogram (ECG), echocardiogram or MUGA scan. These
tests may be done to assess how well your heart is working.
24-hour urine collection to assess how well your kidneys are working.
Please contact our helpline if you would like further information about blood tests or scans.
Burkitt lymphoma
Figure 2: Lumbar puncture
Staging of Burkitt lymphoma
The ‘stage’ of your lymphoma shows which parts of your body have been affected. It is based
on the examination by your doctor and the results of any tests you have had. The stage of
your lymphoma will help the doctors to decide on the best treatment for you.
Burkitt lymphoma may be classed as being ‘favourable’ or ‘unfavourable’. Do not be alarmed
if you have ‘unfavourable’ disease, which is the case for most adults. The intensive treatments
commonly used for Burkitt lymphoma are designed for this type of disease. Those with
‘favourable’ disease may need slightly less treatment.
Most lymphomas, particularly in adults, are staged according to the following system, known as
the ‘Ann Arbor system’.
Stage I
One group of lymph nodes affected
Stage II
Two or more groups of lymph nodes affected on one side of the diaphragm (see
Figure 1)
Stage III Lymph nodes affected on both sides of the diaphragm
Stage IV Lymphoma is found either in organs outside the lymphatic system or in the
bone marrow
Using this system, your Burkitt lymphoma will be classed as favourable if three of the following
apply to you:
your LDH is normal
your performance status is good (meaning you are up and about and able to carry out
light work)
your Ann Arbor stage is I or II
your lymphoma is in no more than one extranodal site.
Burkitt lymphoma
Burkitt lymphoma is sometimes staged using the Murphy staging system instead, which is more
often used for lymphomas in children.
Stage I
One group of lymph nodes or a single extranodal site affected, but not in the
chest or abdomen
Stage II
Two or more groups of lymph nodes or two extranodal sites or one extranodal
site and the nearby lymph nodes affected on one side of the diaphragm
(see Figure 1)
Or tumour in the bowel, whether or not the nearby nodes are affected
Stage IIR
The lymphoma was in the abdomen but has been completely removed by
Stage III
Lymph nodes or extranodal sites affected on both sides of the diaphragm
Or any tumours that started within the chest or alongside the spine
Or large tumours that started within the abdomen
Stage IIIA The lymphoma is in the abdomen only but cannot be removed by surgery
Stage IIIB
Three or more organs within the abdomen involved
Stage IV
Lymphoma in the bone marrow* or in the central nervous system
* You will be regarded as having acute lymphoblastic leukaemia if more than a quarter of your
bone marrow is replaced by lymphoma cells. But you will still receive the same treatment as
other patients with Burkitt lymphoma.
Using this staging system, your Burkitt lymphoma will be classed as favourable if it is stage I or
IIR and your LDH is also normal.
How is Burkitt lymphoma treated?
Burkitt lymphoma is best treated with intensive chemotherapy, as long as your doctors
think you are fit enough for this. Because of the type of chemotherapy needed and the fact
that Burkitt lymphoma is uncommon, not all hospitals will offer this treatment. You may be
transferred to a specialist centre where they are more used to treating this type of lymphoma.
Being diagnosed with Burkitt lymphoma and told about the treatment you will need can be
very frightening. It is important to bear in mind that Burkitt lymphoma generally responds very
well to treatment. In fact, if you are able to complete all your treatment you have a very good
chance of being cured. Do talk to someone if you find things difficult or scary, especially at
the start. Your hospital team will understand how you feel and will want to do all they can to
support you.
You may already be in hospital when you are diagnosed because of your symptoms. If not, you
will be admitted to hospital very quickly once the diagnosis has been made. This is because
treatment for fast-growing Burkitt lymphoma needs to be started quickly before it can make
you more unwell. Being an inpatient means any tests and procedures can be done promptly so
that you are quickly ready to start your treatment.
Burkitt lymphoma
What is chemotherapy and how is it given?
Chemotherapy means treatment with drugs or medicines. For Burkitt lymphoma, it involves
giving a sequence of several different drugs that target the cancerous cells in different
ways. The doses of the drugs used are high compared with those for many other types
of lymphoma. This increases the chances of killing most of the lymphoma cells as quickly
as possible. In addition, almost all patients with Burkitt lymphoma receive the antibody
treatment rituximab (MabThera®) with their chemotherapy. Rituximab therapy is described in
more detail on page 9.
The chemotherapy typically used for Burkitt lymphoma is given intravenously (into a vein),
usually as an infusion (a drip). Some of the drugs need to be given repeatedly for several days
or are infused over a long period of time. Because of this, it can only be given to inpatients. In
Burkitt lymphoma specific treatment is also needed to target the CNS (the brain and spinal
cord). Some of the intravenous drugs do this, but usually drugs are given directly into the
cerebrospinal fluid too. This is known as intrathecal chemotherapy (see page 8).
As well as the infusions of drugs, you will need lots of fluids to keep your kidneys working
well. You will probably receive the drug rasburicase (Fasturtec®) too. This new drug has
made serious kidney problems much less common in people having chemotherapy for Burkitt
lymphoma. You will also need to have regular blood tests to carefully monitor your kidneys,
liver and blood cell counts. To make all this treatment easier, your doctors will probably
recommend you have a tunnelled central line put in (see below) or you may be offered a
PICC line (peripherally inserted central catheter) as an alternative (see page 8).
It is likely your treatment will take several months and you should expect to spend days or
even weeks at a time in hospital. If you are having intensive chemotherapy, you may get a
number of different side effects (see page 8). There are lots of things that can be done to help
prevent or relieve these. Do let people know how you are feeling and ask questions if you
don’t know what is happening.Your hospital team will be there to support you through your
What is a tunnelled central line?
A central line is a soft plastic tube (or catheter) that is placed into a large vein near your
heart. Fluids can go in through it and usually blood samples can be taken out without the
need for repeated needles. A tunnelled central line is usually positioned on your upper
chest with part of the line running through a tunnel under your skin. This reduces the risk
of infection and allows the line to stay in for a longer time. You may hear these called a
Hickman® or Groshong® line.
The line is inserted during a small operation that usually requires a local anaesthetic. It will be
held in place, at least at first, by a few small stitches and will be covered with a clear dressing.
Hopefully it will stay in place for all of your treatment. When you go home, you will be given
instructions about how to care for it. Despite this, central lines can become infected. You
should contact your hospital immediately if you develop a temperature, feel shivery or if the
area around the line becomes red or sore.
Burkitt lymphoma
What is a PICC line?
A PICC line (peripherally inserted central catheter) is a soft plastic tube like a central line but
it is put in through a vein in your arm at the level of your elbow. There is no tunnel so it may
need to be replaced earlier, but it is generally easier to put in and take out. You should contact
your hospital immediately if you develop a temperature, feel shivery or if the area around the
line becomes red or sore.
What is intrathecal chemotherapy?
Chemotherapy that is injected directly into the cerebrospinal fluid (the fluid that bathes the
spine and brain) is known as intrathecal chemotherapy. Only a few drugs can be given in this
way (most often methotrexate and cytarabine).
It is always given by a registrar or consultant during a lumbar puncture (see Figure 2) and a
specialist chemotherapy nurse will also be present. Sometimes a device called an ‘Ommaya
reservoir’ may be put in to avoid the need for lots of lumbar punctures. You may hear this
treatment referred to as ‘CNS-directed therapy’ or ‘CNS prophylaxis’. Please contact our
helpline if you would like further information about this type of treatment.
What are the side effects of chemotherapy?
The most common side effects of the kind of chemotherapy usually used for Burkitt
lymphoma are:
Low blood counts
-Low white cell count (neutropenia): you will be much more prone to infections; your
hospital team need to know at once if you have any signs of infection.
-Low red cells (anaemia): you may be short of breath, or fatigued; a blood transfusion may
-Low platelets (thrombocytopenia): you are more likely to bleed and your body will be
less able to stop any bleeding; you may be given platelet transfusions to protect you or
to help stop bleeding.
Sore mouth (mucositis) or mouth ulcers: using mouthwashes regularly can help.
Feeling sick or having diarrhoea: medication can often help.
Hair loss: this is temporary; it typically begins within a couple of weeks and usually starts
growing back a month or two after treatment has finished.
Fatigue: this is very common; you may find it hard to concentrate or make decisions; you
may even feel too tired to do simple things like watch TV or read.
Damage to nerves (peripheral neuropathy): this commonly affects the nerves
in your hands and feet; you might notice pins and needles, pain, numbness, clumsiness,
problems with balance, increased sensitivity to heat or constipation.
There are also a number of side effects that might affect you in the long term if your
lymphoma is cured. These include reduced fertility and an increased risk of second cancers
and heart disease. Your doctors will discuss with you what your risks are and what can be
done to help.
Burkitt lymphoma
What other therapies might I also have?
Rituximab (MabThera®) is now almost always added to chemotherapy for Burkitt lymphoma.
It is a form of antibody therapy that targets CD20, a protein on the surface of B-lymphoma cells.
It has been shown to improve success rates for treatment of many types of lymphoma. You
may be asked to consider taking part in a trial to prove that it makes the treatment for Burkitt
lymphoma work better too. Please contact our helpline if you would like more information
about rituximab or clinical trials.
Along with your chemotherapy you may be given a number of other drugs including:
mesna and folinic acid, to protect your body from later drug effects
anti-sickness medication (antiemetics)
rasburicase (Fasturtec®) initially and allopurinol in later cycles, to protect your kidneys,
particularly when many lymphoma cells are killed very quickly at the start of treatment
prophylactic (preventive) antibiotics and antifungals, to reduce the risks of infection
growth factors (G-CSF), to help your bone marrow recover quickly.
Specific chemotherapy regimens
One combination commonly used for adults in the UK is R-CODOX-M, which may be
combined with R-IVAC. These initials refer to the names of the individual drugs. R-CODOX-M
is rituximab, cyclophosphamide, vincristine (Oncovin®), doxorubicin and methotrexate. R-IVAC
is rituximab, ifosfamide, etoposide (VP-16 or Vepesid®) and cytarabine (Ara-C). The doses of
cyclophosphamide, ifosfamide, etoposide and cytarabine are each repeated on several days and
the methotrexate takes a full day to give. Other treatments and lots of fluids are also needed to
make sure the drugs do not stay in the body for longer than needed.
R-CODOX-M on its own is used for people with Burkitt lymphoma that is considered
‘favourable’. Basically, this is early-stage Burkitt lymphoma that has not yet had a big impact on
general health (see page 5). The R-CODOX-M cycle is usually repeated three times in all. For
most people, with more advanced-stage Burkitt lymphoma, the combination R-CODOX-M and
R-IVAC is used. Usually two cycles of R-CODOX-M are given, alternating with two cycles of
Another combination regimen that has been widely used for Burkitt lymphoma is
R-hyper-CVAD. This consists of rituximab, frequent small doses of (hyperfractionated)
cyclophosphamide, vincristine, doxorubicin (Adriamycin®) and dexamethasone. This
combination is alternated with high-dose methotrexate and cytarabine. Typically four cycles
of each will be given.
In children and adolescents, several different combinations, which formed part of the LMB96
trial, are used. These include two cycles of COPADM, which consists of cyclophosphamide,
vincristine (Oncovin®), prednisolone, doxorubicin (Adriamycin®) and methotrexate, along
with intrathecal chemotherapy. Typically other chemotherapy regimens, such as CYVE
(cytarabine and etoposide [Vepesid®]) or CYM (cytarabine and methotrexate) are used
after the COPADM depending on how advanced the lymphoma is and how it responds to
early treatment. Recently, trials in this age group have been focused on avoiding unnecessary
treatment to reduce possible long-term side effects.
Burkitt lymphoma
What happens after initial treatment?
A few weeks after you complete your initial chemotherapy, your doctors will perform tests
to check how well your lymphoma has responded. Hopefully you will be in a complete
remission. This means that there are no signs left of your lymphoma.
You will continue to see your specialist or another member of the hospital team regularly.
This follow-up is to see how you are feeling, to monitor your recovery from treatment and
to check there are no signs of the lymphoma coming back. People who have had Burkitt
lymphoma will usually be followed up for several years after the end of their treatment.
To start with, your appointments will be frequent, possibly once a month. This is because
most problems will occur early on after treatment for Burkitt lymphoma. If your disease
has not relapsed after 6 months, it is very likely your treatment has been successful, so your
appointments will be spread out to every 6 months or more.
If you are worried about your health at any time, you do not have to wait for your next
appointment. You should contact your GP or hospital team to discuss your concerns.
Further treatment
If your lymphoma is not in complete remission after your initial treatment or if it comes
back later (relapses), your doctors may offer you further treatment. What can be offered will
depend on what treatment you have already had and how fit you are.
You may be offered high-dose chemotherapy at this stage, which is different from the first
treatment you will have received. This might also be followed by a stem cell transplant, which
allows an even bigger dose of treatment to be given.
Stem cells can be found in the blood and bone marrow and are the source of all blood cells.
Stem cell transplants can be done using either your own cells (an autologous transplant) or
another person’s cells (an allogeneic transplant). They are used to ‘rescue’ the bone marrow
so it can produce new blood cells again after you have received treatment. These procedures
can have a number of risks and will not be suitable for everyone. Your doctors and hospital
team will discuss carefully with you any further treatment, so that you can decide what is best
for you.
Burkitt lymphoma is a rare type of high-grade (fast-growing) B-cell non-Hodgkin lymphoma. It
can affect both children and adults.
The symptoms of Burkitt lymphoma usually come on quickly. They may include swelling of
lymph nodes or other organs, night sweats, weight loss, abdominal pain, sickness, diarrhoea and
shortness of breath.
Burkitt lymphoma can often be cured with intensive chemotherapy. The treatment involves
both intravenous and intrathecal therapy. Typically it will take several months to complete.
You will probably have a tunnelled central line put in so that intravenous drugs and fluids can
be given and blood tests taken. You may get a number of side effects during or after your
Burkitt lymphoma
treatment but you will be given treatments and support to help you cope with these. You
will probably need to spend days or even weeks at a time in hospital. Your hospital team will
explain what you should expect and will be there to support you through your treatment.
We are grateful to Dr Andrew McMillan for reviewing this article. Dr McMillan is a consultant
haematologist at Nottingham City Hospital.
Useful sources of further information about Burkitt lymphoma and
Macmillan Cancer Support
89 Albert Embankment
London SE1 7UQ
0808 808 00 00 (Monday–Friday, 9am–8pm)
Order line for booklets 0800 500 800
via website
CancerHelp UK
The patient information section of Cancer Research UK
0808 800 4040 (Monday–Friday, 9am–5pm)
via website
Selected references
The full list of references is available on request. Please contact us via email ([email protected]
lymphomas.org.uk) or telephone 01296 619409 if you would like a copy.
Swerdlow SH, et al (eds). WHO Classification of Tumours of Haematopoietic and Lymphoid
Tissues. 2008. IARC, Lyon.
Linch DC. Burkitt lymphoma in adults. British Journal of Haematology, 2012. 156: 693–703.
Miles RR, et al. Risk factors and treatment of childhood and adolescent Burkitt lymphoma/
leukaemia. British Journal of Haematology, 2012. 156: 730–743.
Sandlund JT. Burkitt lymphoma: staging and response evaluation. British Journal of Haematology,
2012. 156: 761–765.
Mohamedbhai SG, et al. Rituximab in combination with CODOX-M/IVAC: a retrospective
analysis of 23 cases of non-HIV related B-cell non-Hodgkin lymphoma with proliferation index
>95%. British Journal of Haematology, 2011. 152: 175–181.
Mead GM, et al. A prospective clinicopathologic study of dose-modified CODOX-M/IVAC in
patients with sporadic Burkitt lymphoma defined using cytogenetic and immunophenotypic
criteria (MRC/NCRI LY10 trial). Blood, 2008. 112: 2248–2260.
Patte C, et al. Results of the randomized international FAB/LMB96 trial for intermediate risk
B-cell non-Hodgkin lymphoma in children and adolescents: it is possible to reduce treatment
for the early responding patients. Blood, 2007. 109: 2273–2280.
Burkitt lymphoma
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Produced 11.09.2012
Next revision due 11.09.2014
Burkitt lymphoma