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This information is about a rare type of non-Hodgkin lymphoma| called Waldenström macroglobulinaemia. It may also be called lymphoplasmacytic lymphoma.
There are many different types of non-Hodgkin lymphomas and WM is a rare type. It's more common in people over 65, but it can also affect younger people. It is slightly more common in men than in women.
Non-Hodgkin lymphoma is a cancer of the lymphatic system|. The lymphatic system is part of the body’s immune system and helps us fight infection. It's made up of organs such as the bone marrow, thymus, spleen and the lymph nodes (or lymph glands). Lymph nodes are connected by a network of tiny lymphatic vessels that contain lymph fluid.
There is also lymphatic tissue in other organs, such as the skin, lungs and stomach.
The lymphatic system
View a large copy of the diagram of the lymphatic system|
There are lymph nodes all over the body. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything harmful or that the body doesn't need. This includes bacteria, viruses, damaged cells and cancer cells.
Lymph fluid contains cells called lymphocytes. These are a type of white blood cell that help the body fight infection and disease.
Lymphocytes start to grow in the bone marrow, where blood cells are made. The two main types of lymphocytes are B-cells and T-cells.
B-cells mature in the bone marrow, while T-cells mature in the thymus gland behind the breast bone. When they're mature, both B-cells and T-cells help to fight infections.
Lymphoma is a disease in which either T-cells or B-cells grow in an uncontrolled way.
Some B-cells develop into cells called plasma cells. Plasma cells make antibodies to help fight infections. Antibodies are made from a special type of protein called immunoglobulin.
WM affects B-cells when they are developing into plasma cells. These cells are called lymphoplasmacytic cells, which is where the name lymphoplasmacytic lymphoma comes from.
In WM, the bone marrow produces abnormal B-cells. Although they are of no use to the body, these cells keep being made. As their numbers increase they can build up in lymph nodes, the spleen and other organs.
These abnormal cells produce large amounts of the protein immunoglobulin macroglobulin (IgM). Macroglobulinaemia (the M in WM) just means there is more lgM in the blood than normal.
Large amounts of IgM in the blood can cause the blood to become thicker than normal. This is known as hyperviscosity.
The causes are unknown. WM is not infectious and cannot be passed on to other people.
WM usually develops over a long period of time. Some people have no symptoms and it is found by chance, following a blood test for some other reason.
Symptoms are often due to the lymphoma cells filling up the bone marrow so that it cannot produce enough normal blood cells.
The most common symptoms are:
If there are high levels of the IgM protein in the blood, some people may have symptoms caused by the blood becoming too thick and not flowing freely (hyperviscosity). These can include changes in vision, headaches, hearing loss or confusion. Sometimes people develop numbness or tingling in their hands and feet| or problems with their balance. This is due to nerve damage caused by the abnormal protein in the blood.
These symptoms can also be caused by other conditions, but they should always be checked out by a doctor.
Your GP will examine you and do a blood test. Depending on the result, you may then be referred to a hospital for specialist advice or treatment. The doctor at the hospital will ask you about your symptoms and any other health problems you have, examine you physically, and do further blood tests. These tests will show if there are abnormal cells in the blood and will also measure the amount of the IgM protein in your blood.
The doctor may also ask you to have further tests|, such as a bone marrow test and a CT scan.
Your specialist may want to take a sample of marrow (biopsy) to examine under a microscope.
The sample is usually taken from the back of your hip bone (pelvis). You will be given an injection of local anaesthetic to numb the area. The doctor then passes a needle through the skin into the bone and draws a small sample of liquid marrow into a syringe (bone marrow aspirate). After this, they take a small core of marrow from the bone (a trephine biopsy). Both samples will be looked at later under a microscope.
The test can be done on the ward or in the outpatients department. The whole procedure takes about 15-20 minutes. It may be uncomfortable as the marrow is drawn into the syringe, but this should only last for a few seconds.
You may feel bruised and have an ache for a few days after the test. This can be eased with mild painkillers.
A CT scan is a special type of x-ray. It is used to find out if WM has affected lymph nodes or organs, such as the liver.
A number of pictures are taken from different angles and fed into a computer which shows detailed pictures of the inside of the body. This involves lying still for 45-60 minutes.
You may be asked not to eat or drink anything for at least four hours before your appointment. Most people who have a CT scan are given a drink, or an injection into a vein in the arm, about an hour before the scan. This allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes.
Before having the injection or drink it is important to tell your doctor and the person doing the test if you are allergic to iodine or have asthma.
WM often develops slowly. This means that some people may not need treatment straight away, or even for months or years.
Even if you don’t need treatment, you will have regular check-ups at the hospital. Your specialist will examine you and do blood tests to check the levels of your red blood cells, white blood cells and platelets (a full blood count or FBC) and the levels of IgM protein in your blood.
Your doctor will consider starting treatment if:
The main treatment for WM is chemotherapy (anti-cancer) drugs. Some symptoms can also be improved with plasma exchange and blood transfusions|.
Chemotherapy| is the use of anti-cancer (cytotoxic) drugs. It works by destroying abnormal B-cells. This controls the disease and reduces symptoms. Chemotherapy may be given as several sessions of treatment over a specific length of time. Usually there is more than one type of chemotherapy that may be helpful. Your doctor will discuss the different options with you. You may be asked to have your chemotherapy as part of a research trial|.
Your doctor will give you advice about how to cope with any side effects you have from the treatment, and can give you medicines to reduce them if necessary.
Chlorambucil| is taken as a tablet and is usually given daily for the time recommended by your specialist. It should be kept in the fridge.
Side effects are usually mild. One of the common side effects is a temporary reduction in blood cell production. This can result in anaemia, risk of bruising or bleeding, and infection|. Any infections will need to be treated with antibiotics, and your blood count will be monitored throughout your treatment. If your temperature goes up or you suddenly feel unwell, even with a normal temperature, contact your hospital or doctor straight away.
You may have some mild nausea and vomiting, but this can usually be controlled with anti-sickness drugs|. Other possible side effects include a loss of appetite|, mouth ulcers|, a skin rash or diarrhoea|.
Fludarabine| is usually taken as tablets but it may be given as an intravenous injection (an injection into a vein). It is taken every day for 3-5 days. The treatment is usually repeated every three to four weeks.
Cladribine| is a similar type of drug to fludarabine. It may be given as an injection into a vein (intravenous) or as an injection just under the skin (subcutaneous) every 6-12 weeks for two courses.
Common side effects include an increased risk of infection. The first sign of infection may be a high temperature or feeling cold and shivery. Any infection needs to be treated immediately with antibiotics. Again, if you have a high temperature or if you suddenly feel unwell, contact your hospital or doctor straight away. You may feel sick or be sick, but this can usually be well controlled with anti-sickness drugs. Other possible side effects include loss of appetite, mouth ulcers, diarrhoea and sometimes constipation|.
Other drugs, such as cyclophosphamide|, and combinations of drugs, for example the CHOP regimen| (cyclophosphamide, doxorubicin, vincristine and prednisolone), may be used in particular circumstances. Your specialist will explain which treatment is appropriate for you.
Steroids| can be used as part of your treatment to help destroy the abnormal lymphocyte cells and make chemotherapy more effective. They're usually given to you as tablets or as a liquid, but it may also be given as an injection into a vein (intravenous).
The side effects of steroids are temporary and will usually go away when your treatment finishes.
Monoclonal antibodies| are drugs that recognise, target and stick to particular proteins on the surface of cancer cells. They can stimulate the body’s immune system to destroy these cells.
A monoclonal antibody called rituximab| (also known as Mabthera®) is sometimes used to treat WM. It's given as a drip into a vein (infusion). Rituximab may be given with chemotherapy and/or steroids.
Some people with WM may have treatments involving using their own stem cells| or stem cells from a donor|. Stem cells are very early blood cells.
There are serious side effects associated with these treatments. They're not suitable for everyone and are not done routinely. Doctors take into account a person's general health and age before recommending them.
Some of your own stem cells can be removed and stored. This allows you to have higher doses of chemotherapy to destroy the lymphoma cells. After the chemotherapy your stem cells are returned to you by a drip (like a blood transfusion) to help your blood cells recover from the effects of chemotherapy. The stem cells make their way to the bone marrow and start making new blood cells. This is called an autologous transplant.
Occasionally stem cells from another person (a donor) may be used. This is called an allogeneic transplant.
Plasma exchange| (or plasmapheresis) is a process carried out to ‘thin’ the blood if high levels of IgM are making it thick. The procedure can take 2-4 hours and is not painful.
You will lie on a bed with a plastic tube (cannula) placed in each arm. The cannula is connected to a machine called a cell separator, which your blood is circulated through, a small amount at a time. This machine separates the plasma (which contains the IgM protein) from the blood cells. The normal blood cells, along with a plasma substitute (for the plasma that is removed), are then returned to your body through the drip in your other arm.
The number of plasma exchanges you have will depend on the amount of IgM protein in your blood, your symptoms and your response to treatment such as chemotherapy.
Plasma exchange may be done once a month if it is the only treatment being given. If there are extremely high levels of IgM protein in the blood, it can be done as an emergency treatment (daily for 1-2 days) before chemotherapy starts.
Rarely, people who have an enlarged spleen may have it removed with surgery|. Removing the spleen before chemotherapy starts may help to relieve symptoms in some people. Your doctor will discuss the operation with you.
After you have had your spleen removed, you may be more prone to certain infections|. To prevent this, your doctor will prescribe different vaccinations and antibiotics for you. They will tell you how long you will have to take antibiotics. It may be for several years or for life.
Once your course of treatment has finished, you will continue to have regular blood tests and check-ups. If you feel unwell at any time between these appointments, it is important to let your doctor know as soon as possible.
New treatments for WM are being researched all the time. Your doctor may invite you to take part in a clinical trial| to compare a new treatment against the best available standard treatment.
Your doctor must discuss the treatment with you and have your informed consent before entering you into a trial.
Before any trial is allowed to take place, it must be approved by a research ethics committee, which protects the interests of those taking part. You may decide not to take part or to withdraw from the trial at any stage. You will then receive the best standard treatment available.
Everyone has their own way of dealing with their illness and the different emotions| they experience. You may find it helpful to talk things over with family and friends or your doctor or nurse.
You can also contact our cancer support specialists| or the organisations listed below for more information and support.
IWMF| is an international support group for Waldenström macroglobulinaemia.
Leukaemia & Lymphoma Research| provides written information on Waldenström macroglobulinaemia.
This section has been compiled using information from a number of reliable sources, including:
For answers, support or just a chat, call the Macmillan Support Line free (Monday to Friday, 9am-8pm)
If you have any questions about cancer, need support or just want someone to talk to, ask Macmillan.