If you have been diagnosed with WM you might have no treatment at all for a while (see below) or you might have one or more of the following treatments:
- chemotherapy drugs
- monoclonal antibodies
- stem cell transplant
You might also need other treatments if you have thickening of your blood, if your blood counts are low, or if you have side effects from the chemotherapy drugs. These additional treatments are called ‘supportive’ treatments.
The treatment you will be given will depend on your particular circumstances. It will depend on the results of all the tests, on your symptoms and your age and general health.
WATCH AND WAIT?
The first response of most people diagnosed with cancer is to look for immediate treatment. This is not always needed as WM is slow moving and may take years to develop, or in some cases never. Most symptoms are not usually life threatening. Hence patients may be put on 'watch and wait'. This decision is determined by the relative progression of the disease and the severity of the patient’s symptoms. When a patient is first diagnosed it is important to begin recording blood test values and symptoms to establish a baseline. Its usual also to have an intial scan to see if other organs are involved.
As additional laboratory tests are performed and patient symptoms are monitored, a pattern will become evident which will indicate a rate of disease progression. In many WM cases, there will be very little change in status from visit to visit. For these patients a strategy of W&W may be an option. Many patients keep a record of their key tests and relate them to their general well being and symptoms.
W&W avoids the use of treatments which could have side effects until treatment is absolutely necessary. The question is: “By delaying treatment am I allowing WM to become more difficult to treat in the future or am I allowing symptoms to progress to the point that they cannot be easily managed?” This question can only be answered by you and your specialist after a review of your individual test results and symptoms.
Not a treatment as such, but for patients disgnosed with high levels of IgM producing symptoms, plasmapheresis is a rapid way of reducing them. In some ways like kidney dialysis, the patient is connected by a line in each arm to an apheresis machine for 2-3 hours, the heavier IgM is centrifuged off and blood returned. There are very few side effects. This gives immediate relief typically lasting for a month or two and a breathing space whilst doctor and patient negotiate the best treatment. The photos show a day patient at the National Blood Transfusion centre at Bristol. The nurse is programming the apheresis machine.
Plasmapheresis at the Bristol National Blood Transfusion Centre
Chemotherapy (treatment with anti-cancer drugs) can be given to destroy the abnormal B cells. Several chemotherapy drugs have been proved to be effective in WM. Some of these drugs are given intravenously (into a vein through a plastic tube called a cannula), some are taken by mouth in tablet or liquid form and some are given subcutaneously (injected just under the skin).
When treating WM, two or more drugs with different actions may be used together. These drug combinations are more effective than single drugs in attacking the abnormal B cells. The drugs are given in cycles over a period of a few months, meaning that you will have treatment some weeks but not others. This allows healthy blood and bone marrow cells to recover between treatments.
There are a number of different combinations and doses of drugs used. Your treatment will beindividually tailored by your medical team to ensure that you are having the best and most suitable course of treatment for you. The combination which is chosen will depend on your age, what symptoms you have, how severe your symptoms are and your blood counts. It will also depend on what treatments you might possibly require in the future (certain drugs are best avoided, for example, if you might need a stem cell transplant later on).
The different chemotherapy treatments commonly used to treat WM are:
Chlorambucil: Chlorambucil is a commonly used chemotherapy drug and is taken in tablet form. It is usually taken daily for a period of time specified by the doctor. Its side effects are normally mild.
Fludarabine and Cladribine: Fludarabine can be given as tablets or intravenously, usually every day for 5 days. Treatment is usually repeated every 3–4 weeks for 6–8 months. This chemotherapy produces a more rapid response than chlorambucil, but its side effects can be more severe.
Cladribine is a similar type of drug to Fludarabine and has similar side effects, but it is given using a different schedule. It is can be given intravenously or subcutaneously every 6–8 weeks for two courses.
Chemotherapy bay at University College Hospital
Other chemotherapy drugs: Other types of chemotherapy drugs such as cyclophosphamide, doxorubicin and vincristine may be used in certain situations. These drugs are typically given in combinations known as ‘CHOP’ or ‘CVP’, in which the ‘P’ stands for prednisolone, a type of steroid that is given at the same time as the chemotherapy drugs (see below). These combinations are usually given every 3 weeks for six cycles.
Other combinations of chemotherapy drugs and other agents may also be used – your medical team will discuss these with you.
To an extent there is a degree of 'postcode prescribing' in the NHS at present, particularly in the availability of Rituximab based chemotherapies- you are strongly advised to ask specifically what is available in your area, and compare with other patients online.
Potential side effects of chemotherapy
All chemotherapy drugs have potential side effects. These vary from patient to patient and the different drugs used. It is important to remember that most side effects are manageable and will resolve once treatment is finished.
Common side effects of chemotherapy include nausea, loss of appetite, change in bowel habit, hair thinning or loss, and an increased tendency to develop infections, bruising and bleeding.
Steroids are used to reduce inflammation in many medical conditions. In WM they also appear to cause a process called ‘programmed cell death’. This means that steroids can trigger the destruction of the abnormal B cells. When used in combination with chemotherapy, steroids can make your lymphoma more responsive to the chemotherapy drugs and therefore make the treatment more effective. In the treatment of WM, steroids are usually given by mouth in tablet or liquid form, but they can also be given intravenously.
Potential side effects of steroids
Side effects of steroids do vary. It is important to remember that each person’s reaction to steroids can be different and that side effects, if any, are temporary and should resolve when the steroids are stopped. Common side effects of steroids include indigestion, increased blood sugar, increased blood pressure, increased risk of infection, increased appetite, mood changes and weakness due to muscle wasting.
A key decision factor is the possibillity of a stem cell transplant as a last resort - some chemotherapies may make the collection of your own stem cells difficult in future.
Monoclonal antibody treatment
Monoclonal antibodies are drugs that recognise, target, and stick to certain proteins on the surface of some cancer cells. This marks out the cells for destruction by the body’s immune system. A monoclonal antibody called Rituximab is commonly used in the treatment of WM, alongside chemotherapy and/or steroid treatment. Such combinations are known by their initial letters eg R- CHOP.
Rituximab is given slowly into your vein the first time it is given and then it is given a more quickly on subsequent infusions if it is well tolerated.
Potential side effects of monoclonal antibodies
You can experience flushes, sweats, a fast pulse rate and a decrease in blood pressure during the infusion of Rituximab, which is why the first infusion is given slowly. Most people tolerate this drug without problems, however.
Less commonly used drugs and new drugs
Other drugs that have been shown to be effective in clinical trials, either alone or combined with other drugs, include bortezomib, bendamustine, thalidomide and ofatumumab. Although effective, these drugs are still being tested in clinical trials and are not yet in general use. Permission to use these drugs usually needs to be specifically sought from the regulatory bodies by the doctor before they can be used. New drugs are becoming available all the time and continue to be tested in clinical trials. If a PCT refucses initially an expensive treatment the new Cancer Drugs Fund may be an alternative- ask your specialist.
Stem cell transplantation
This is also known as 'peripheral blood stem cell transplantation'. This type of treatment is sometimes considered for younger (under 65 possibly), fitter, people with WM who are able to tolerate the intensity of the treatment. Stem cells can be obtained either from your own blood or from a donor and they are infused (dripped slowly through a cannula into your vein) after you have had a course of high-dose chemotherapy.
Autologous stem cell transplant: When you act as your own donor, this is known as an ‘autologous’ transplant. In this form of transplant your own stem cells are collected. Having the cells collected is a bit like what happens when you give blood. These cells are stored until after you have had your course of high-dose chemotherapy to kill any remaining lymphoma cells. Your stem cells are then returned to you. They make their way to your bone marrow, where they form new blood cells to restore your bone marrow to normal function.
Collection of stem cells
Stem Cell Harvest
2 million stem cells!
This form of stem cell transplant is not curative, but it can lead to you having a long-lasting remission, meaning that the disease can stay at a very low level for quite a long time before further treatment is needed. Typicall the whole process will last two weeks in hospital.
Allogeneic stem cell transplant: When the stem cells come from another person this is known as an ‘allogeneic’ transplant. The donor might be a brother or sister or someone not related to you but whose tissue type matches yours. In this type of stem cell transplant, the donor’s stem cells produce donor bloodcells in your bone marrow, and these cells can directly fight against any leftover lymphoma cells so that the disease is treated using the donor’s immune system as the weapon.
While this form of transplant can offer the possibility of cure for some people with WM, it is a more hazardous procedure than an autologous transplant and your general health has to be good before you would be considered for it. You would need to think about the risks and benefits very carefully before embarking on this form of treatment. Your medical team would discuss this option in detail with you if they felt you could benefit from it.
Where to be treated?
To some extent there is a postcode lottery in these difficult economic times between different hospitals, and you may find that new treatments available in the USA or EU are not available in the UK or one or more of its component countries. It's usual for teaching hospitals to have greater flexibility in offering the latest treatment, and they are more likely to run trials of new therapies.
WMUK works with other organisations to try to promote new treatments in the UK.