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This information is about a rare type of non-Hodgkin lymphoma, known as Waldenström’s macroglobulinaemia. It may also be called lymphoplasmacytic lymphoma or immunocytoma. It should ideally be read with our general information about non-Hodgkin lymphoma.
Waldenström's macroglobulinaemia (pronounced mac-row-glob-you-lin-eem-ia) is a rare type of non-Hodgkin lymphoma (NHL): a cancer of the lymphatic system. The lymphatic system is part of the body’s immune system and helps us fight infection. It is a complex system made up of organs such as the bone marrow, thymus, spleen, and the lymph nodes (or lymph glands). These are connected by a network of tiny lymphatic vessels that contain lymph fluid. Lymphatic tissue can also be found in other organs, such as the lungs, stomach and skin.
There are lymph nodes all over the body. As lymph fluid flows through the lymph nodes, the nodes collect and filter out anything that the body does not need, or that could harm the body. This includes bacteria, viruses, damaged cells, and cancer cells.
There are two main types of lymphocyte: B-cells and T-cells. Most lymphocytes start growing in the bone marrow. The B-cells continue to develop in the bone marrow. The T-cells go from the bone marrow to the thymus gland (behind the breast bone) and mature there.
When they are mature, some B-cells develop into specialised cells called plasma cells which make, store, and release antibodies to help fight infections and harmful substances. Antibodies are made from a special type of protein called immunoglobulin. This can change its structure in order to recognise and fight particular infections.
In Waldenström’s macroglobulinaemia, lymph cells multiply out of control. They go into the bone marrow, lymph nodes, and spleen, and produce large amounts of a particular antibody called IgM. A large amount of IgM in the blood causes thickening (hyperviscosity) of the blood.
There are more than 20 different types of NHL, and Waldenström’s macroglobulinaemia is a rare type, accounting for about 1 in 50 of all cases. It occurs mostly in people over the age of 65, but can occur in younger people, and is slightly more common in men than in women.
The causes are unknown. Waldenström’s macroglobulinaemia, like other cancers, is not infectious and cannot be passed on to other people.
Waldenström’s macroglobulinaemia normally develops over a long period of time. Sometimes people have no symptoms and it is found by chance, following a blood test for some other reason.
Some people go to their doctor with vague symptoms such as weakness, tiredness, and weight loss. Some people may look pale due to a lack of red blood cells (anaemia). Some people have a tendency to bruise more easily due to impaired platelet function. Occasionally, people may be more unwell with these symptoms.
Some people may also develop sight problems, headaches, hearing loss, or confusion due to thickening of the blood (hyperviscosity) caused by high levels of IgM antibody. These symptoms can be caused by a number of different conditions other than Waldenström’s macroglobulinaemia, but any symptoms that get worse or last for a few weeks should always be checked by your doctor.
Some people with Waldenström’s macroglobulinaemia will develop enlargement of particular lymph nodes, or an enlarged spleen (known as splenomegaly). Some people develop an enlarged liver: this is known as hepatomegaly.
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 take your full medical history, carry out a physical examination, and take urine samples and further blood tests. The blood tests will show the number of abnormal cells present and the amount of the IgM antibody in your blood. The doctor may also ask you to have further tests, such as a bone marrow test, an ultrasound, or a CT scan.
Bone marrow sample The hospital doctor may want to take a sample of bone marrow to examine under a microscope (biopsy). This helps to give more specific information about the condition. The sample is usually taken from the hip bone, or occasionally from the breast bone. Before the test the area is numbed, using a local anaesthetic. A needle is then passed gently through the skin, muscle, and bone into the bone marrow, and a small amount is drawn out using a syringe. The test takes about 15 minutes and may be painful for a few minutes. You may need to take painkillers for a few days afterwards.
Ultrasound In this test (which will be done in the hospital scanning department) sound waves are used to make up a picture. The doctor will be able to see if your liver or spleen is enlarged. You will be asked to lie on your back and a special gel is spread over your abdomen. A small probe is rubbed over the area. The echoes are converted into a picture using a computer. It is a totally painless procedure that takes about 15–20 minutes.
CT scan A CT scan is a special type of x-ray. 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 about 10–30 minutes.
For practical purposes, non-Hodgkin lymphomas are divided into two groups: low- and high-grade. Low-grade lymphomas are usually slow-growing, and high-grade lymphomas tend to grow more quickly.
Waldenström’s macroglobulinaemia is a low-grade lymphoma and tends to develop very slowly, so it is known as a chronic condition.
Waldenström’s macroglobulinaemia is a slowly progressing condition. This means that treatment may not be needed straight away, and some people may never need treatment. If you do not need treatment, the doctor will still check your general condition regularly, and arrange for frequent blood tests.
Your doctor will consider starting treatment if you begin to get symptoms, or if your blood results show an increasing antibody level, or low levels of red blood cells (anaemia). When treatment is needed, your doctor is most likely to suggest treatment with chemotherapy drugs. Some symptoms can also be improved with plasma exchange (see below) and blood transfusions. Surgery to remove an enlarged spleen may sometimes be used.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs, which work by stopping the plasma cells from multiplying. This controls the disease and reduces any symptoms. Chemotherapy will be stopped once this is achieved, and your doctor may discuss another course of treatment with you if the disease shows signs of developing. Your doctor will give you advice about how to cope with any side effects you have from the treatment, and can give medicines to reduce them if necessary.
These are the most common chemotherapy drugs used for Waldenström’s macroglobulinaemia:
Chlorambucil (claw-ram-bu-cil) is taken as a tablet and may be given daily for the time recommended by your specialist. Side effects include a temporary reduction in blood cell production that can result in anaemia, risk of bruising or bleeding, and infection. Any infection will need to be treated with antibiotics, and your blood count will be monitored throughout your treatment. Mild nausea and vomiting may occur and are usually well controlled with anti-sickness drugs. Other possible side effects include a loss of appetite and, occasionally, changes to the lungs, skin rashes, mouth sores and ulcers, diarrhoea, and temporary changes in liver function.
Fludarabine (flu-dar-a-been) is given as an intravenous injection (an injection into a vein), usually each day for five days. This may be repeated monthly. Fludarabine may also be taken as a tablet. Common side effects include a high temperature and infection, which need to be treated with antibiotics. Mild nausea and vomiting may occur but this is usually well controlled with anti-sickness drugs. Other possible side effects include loss of appetite, mouth sores and ulcers, diarrhoea or constipation.
Cladribine (clad-ri-been) is either given by a drip (intravenously), or as an injection under the skin every day for 5–7 days, and may be repeated monthly. Common side effects include a high temperature and infection, which will need to be treated with antibiotics. Other possible side effects include headaches, weakness, skin rashes, and muscular aches.
Other drugs and combinations of drugs may be used in particular circumstances, such as:
Plasma exchange (also known as plasmapheresis) is a process carried out to ‘thin’ the blood if high levels of IgM antibody are making the blood thicken. You will lie on a bed with a drip placed in each arm which will be attached to a machine for 1–3 hours. Your blood is circulated through the machine and it separates the plasma fluid (which contains the IgM antibodies) from the blood cells. The blood cells (red and white blood cells and platelets), along with a substitute for the plasma, are then returned to your body. This process can be repeated as often as your doctor feels is necessary.
Some people who have an enlarged spleen find that removing the spleen and having chemotherapy helps to relieve symptoms. Your doctor will discuss the operation with you.
Following removal of the spleen, you may be more susceptible to certain infections. In order to prevent this, various vaccinations and antibiotics will be prescribed for you. Your doctor will tell you how long you will have to take antibiotics: it may be for several years or it may be for life.
Another treatment that has been tried for some people with Waldenström’s macroglobulinaemia is a monoclonal antibody called rituximab (ri-tucks-i-mab), also known as Mabthera®). Monoclonal antibodies are drugs that recognise, target, and stick to particular proteins on the surface of cancer cells, and can stimulate the body’s immune system to destroy these cells.
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.
Research into treatments for Waldenström’s macroglobulinaemia is ongoing and advances are continuously being made. Cancer doctors use clinical trials to assess new treatments. Before any trial is allowed to take place, it must have been approved by an ethics committee, which checks that the trial is in the interest of patients.
Your doctor must discuss the treatment with you so that you have a full understanding of the trial and what it involves. You may decide not to take part, or to withdraw from a trial, at any stage. You will then receive the best standard treatment available.
The need for practical and emotional support will of course be individual. For some people with Waldenström’s macroglobulinaemia, life may seem largely unaffected; for others the diagnosis of cancer may be a cause of great fear and distress. If you would like to discuss the condition, its treatment, or the practical and emotional problems of living with Waldenström’s macroglobulinaemia, please contact our cancer support service.
This section has been compiled using information from a number of reliable sources, including:
For further references, please see the general bibliography.
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