This information has been prepared to help you understand more about multiple myeloma.
Many people feel understandably shocked and upset when told they have multiple myeloma. This information is intended to help you understand the diagnosis and treatment of the disease.
We cannot advise you about the best treatment for you. You need to discuss this with your doctors. However, we hope this information will answer some of your questions and help you think about the questions you want to ask your doctors or other health carers.
What is blood?
Multiple myeloma is a cancer affecting plasma cells, which are a type of white blood cell.
Blood is pumped around your body to supply oxygen and nutrients to your tissues and take away waste products. It is made up of:
- red cells – carry oxygen from your lungs to the rest of your body
- white cells – fight infection
- platelets – form clots to stop bleeding
The different kinds of blood cells are made by the bone marrow (the spongy part in the centre of bones). The bone marrow contains stem cells, which are primitive blood cells that develop into either red cells, white cells or platelets. When a stem cell matures, in most cases it is released into the bloodstream.
Plasma cells are part of the immune system and help to fight infection by making proteins called antibodies or immunoglobulins. Antibodies attack and kill bacteria and viruses that have infected the body.
What is multiple myeloma?
Myeloma is a cancer of plasma cells. It starts in the bone marrow, where large numbers of abnormal plasma cells called myeloma cells are made. These myeloma cells multiply abnormally, without any proper order, forming collections known as tumors that accumulate in the bone marrow and on the surfaces of different bones in the body. Because bone marrow is found in multiple areas of the body (e.g. in the spine, skull and pelvis), the disease is often called multiple myeloma.
Myeloma cells divide and expand within the bone marrow, crowding out the rest of the bone marrow so it can’t make enough healthy blood cells.
Myeloma cells make one kind of antibody (also known as an immunoglobulin) — known as M-protein, monoclonal protein or paraprotein. This antibody is faulty and has no useful function. It reduces the production of normal antibodies and weakens the immune system.
M-protein is in the blood of most people with myeloma. Part of this antibody is found in the urine of some patients, where it is called Bence Jones protein.
Types of multiple myeloma?
There are different types of myeloma, which are classified according to the type of M-protein present. This is called an immunoglobulin or antibody. The structure of each immunoglobulin is made up of two heavy chains and two light chains.
Light chain myeloma – About one in five (20%) people with myeloma produce light chains only. This is called the light chain or Bence Jones protein. The light chains show up in the urine and can be measured in the blood using a test called the serum free light chain assay.
Non-secretary myeloma – In less than two out of 100 (2%) people with myeloma, little or no immunoglobulin is produced, making diagnosis more difficult. In most of these people, the serum free light chain assay can detect small amounts of light chains, but heavy chains are not present.
There are also different stages of myeloma, which indicates the amount of myeloma present and can help determine a person’s prognosis. Myeloma can spread from the bone marrow into the bone itself.
How common is it?
Multiple myeloma is not a common disease. It represents only 1% of all cancer diagnoses in Australia. 428 people were diagnosed with myeloma in NSW in 2005.
Myeloma usually occurs in people aged 60 and over. It is rare in people under 40. The disease is becoming more common in the elderly, which is partly explained by the ageing population. It is found more often in men than women.
What is the cause?
The cause of multiple myeloma is unknown. However, it is more common in older people, men and some racial groups (such as African-Americans).
Increased risk for myeloma has also been linked to:
- certain chemicals (e.g. agricultural chemicals, dioxins)
- viruses (such as HIV)
Myeloma is not hereditary, which means it does not run in families. It is rare for more than one person in a family to be affected. However, there is an increased risk for developing multiple myeloma among people who have a family history of other blood and lymphatic cancers.
What are the Symptoms
Multiple myeloma can cause a range of symptoms because of its effects on the bone marrow, bones and kidneys.
- bone pain or a broken bone that has not been caused by an obvious injury
- kidney problems caused by the excess amounts of paraprotein produced by the myeloma cells. This can cause tiredness and anaemia
- frequent infections or an infection that is difficult to shake off
- tiredness, shortness of breath or a racing heart caused by a low level of red blood cells. This is called anaemia
- heavy nosebleeds or easy bruising due to having fewer platelets
- feeling sick or drowsy because of too much calcium in the blood (hypercalcaemia)
- abnormal blood counts as abnormal plasma cells may stop the bone marrow from making enough normal blood cells.
Another less common symptom of myeloma is weight loss.
If a large amount of M-protein is present, it can damage the kidneys as it passes through them into the urine. Kidney failure and weight loss are less common symptoms of myeloma
Some people with multiple myeloma also have a condition called amyloidosis. This is a build-up in the body of a fibrous protein called amyloid tissue, which can sometimes affect the functioning of different organs. In many people the condition does not cause any symptoms.
Different tests are needed to diagnose multiple myeloma. The tests may be uncomfortable, but they will enable your specialist, usually a haematologist, to determine the type of myeloma you have, how it is affecting you, and the best treatment plan for you.
A number of blood tests are used to diagnose myeloma. The main blood test is called serum paraprotein and will show the amount and type of paraprotein in your blood. Changes in the level of paraprotein indicate changes in the activity of the myeloma.
You will also have a full blood count done, as well as other blood tests to check for anaemia, too much calcium in the blood (hypercalcaemia), and how your kidneys are working.
A urine test is done to check for the Bence Jones protein, which is a part of the M-protein antibody. It is found in around 30% of people with myeloma. You may have to collect your urine in a container for 24 hours. Not everybody with M-protein in the blood or urine has multiple myeloma. Other conditions where M-protein is present include inflammatory diseases, chronic infection, and a condition called MGUS (Monoclonal Gammopathy of Undetermined Significance). People with MGUS do not have symptoms and the majority remain well for years, but some may develop myeloma or related diseases in later years.
Additional tests may be conducted to monitor the effect of the myeloma on your bones.
X-rays take pictures of the inside of your body. The procedure is painless. Bone x-rays (skeletal surveys) are needed to find any bone damage caused by the myeloma cells. X-rays are usually taken of your head, spine, ribs, hips, legs and arms. Areas of bone damage show up on x-ray film as black shaded areas and are known as lytic lesions.
A CT scan uses x-rays to take cross-section pictures of the inside of your body. This test is used to review a part of the body in more detail or to identify areas of bone damage that may not show up on a standard x-ray.
Before the test, a special dye may be injected into a vein, probably in your arm, to help make the pictures clearer. This may make you feel hot all over for a few minutes. If there is a risk of allergy to the dye, it won’t be used.
You will lie flat on a table while the CT scanner, which is large and round like a doughnut, moves around you and takes pictures. The scan is painless and takes between 10 and 30 minutes. You are able to go home as soon as your scan is done.
A MRI (magnetic resonance imaging) uses a combination of magnetism and radio waves to build up detailed cross-sectional pictures of your body.
You will lie on a table that slides into a metal cylinder. Some people find lying in the MRI scan cylinder noisy and claustrophobic. If you feel uncomfortable in confined spaces, let your doctor or nurse know as they may be able to give you medication to help you feel more relaxed. The MRI can sometimes find myeloma in the bone and outside the bone.
A bone marrow biopsy is used to examine cells from the marrow. This procedure is done under a local or general anaesthetic. A thin needle is used to remove a small amount of bone marrow from the pelvis, hip or breastbone.
A biopsy may be uncomfortable. Mild painkillers or a sedative can help ease the discomfort. After a biopsy you may feel a bit drowsy and have some bleeding from the biopsy site.
Each cell in the body contains chromosomes, which are made up of genes. The genes control all activities of the cell. In myeloma there may be changes in the structure of the chromosomes within the myeloma cells, but not the normal cells of the body.
Cytogenetic tests can examine the bone marrow biopsy for changes in the chromosomes, which may help to determine treatment and predict how the myeloma may respond
The results of the blood tests and various scans can help the doctor determine how much cancer there is. This is called staging. Staging the cancer helps your health care team decide what treatment is best for you.
The International Staging System (ISS) is the staging system used for myeloma. It is based on the levels of the blood proteins beta-2 microglobulin (β2M) and albumin:
Stage 1 – when serum albumin is greater than 35g/L and β2M less than 3.5mg/L
Stage II – when serum albumin and β2M both less than 3.5mg/L or when serum β2M between 3.5-5.5 mg/L (irrespective of serum albumin)
Stage III – Serum β2M greater than 5.5mg/L
Prognosis is the expected outcome of a disease. You need to discuss this with your doctor. Only someone who knows your medical history can tell you what to expect and the treatment options that are best for you.
For most people with multiple myeloma, the disease is not curable, but it is treatable and can be controlled.
The typical course of the disease is multiple episodes of disease activity, followed by a plateau phase (when the disease is under control). When the disease comes back (relapses), more treatment will be needed, which may result in another plateau phase. Some people’s prognosis improves significantly with a bone marrow or blood stem cell transplant, but this is only suitable for less than 5% of patients.
Which health professionals will I see?
You will be cared for by a range of health professionals who specialise in different aspects of your treatment. Health professionals who may care for you include:
- General practitioner (GP) – arranges the first tests to investigate your symptoms.
- Haematologist – a doctor who treats people with blood diseases.
- Medical oncologist – prescribes and coordinates targeted therapies and chemotherapy.
- Radiation oncologist – prescribed and coordinates radiotherapy.
- Nurses – support you through all stages of your cancer treatment.
- Cancer care coordinator or clinical nurse consultant (CNC) – supports patients and families throughout treatment and liaises with other staff.
- Dietician – recommends an eating plan to follow while you’re in treatment and recovery.
- Social workers, counsellors, physiotherapists and occupational therapists – link you to support service social worker, and help with emotional, physical or practical issues.
After the diagnosis, the most important decision is whether treatment is needed straight away. Early-stage myeloma, also called asymptomatic or smouldering myeloma, does not need to be treated, as early treatment has not been shown to be of any benefit. People with smouldering myeloma will need regular checkups. This is called active monitoring. Treatment will start if symptoms occur.
The main forms of treatment for multiple myeloma are a combination of chemotherapy, other medications and radiotherapy (x-ray treatment).
Chemotherapy is the treatment of cancer with anti-cancer drugs called cytotoxics. The aim of chemotherapy is to kill cancer cells while doing the least possible damage to healthy cells.
Chemotherapy uses drugs to kill or slow the growth of leukaemia cells. It is usually given by injection into a vein (intravenously), under the skin (subcutaneously) or as tablets.
The most commonly used chemotherapy drugs for multiple myeloma are melphalan and cyclophosphamide. Other drugs are adriamycin, bis-chloronitrosourea (BCNU) and vincristine.
A hospital stay may be necessary for some treatments, but this stay is usually short. Your doctor will tell you about the drugs you are having and how long treatment will last.
Thalidomide and related drugs
Thalidomide is an old drug with new uses. It is now known to have anti-cancer effects, particularly for multiple myeloma. It is thought to work by blocking the blood supply to the cancer and by altering the body’s immune response to myeloma cells. Thalidomide is given as a treatment either on its own, or more commonly, in combination with a steroid and a chemotherapy drug. It is taken as tablets, usually every day.
The steroids used to treat multiple myeloma are called corticosteroids. These drugs are similar to a hormone produced by the body’s adrenal glands.
Corticosteroids are commonly used to relieve swelling and inflammation, and actively kill myeloma cells.
The most commonly used corticosteroids include prednisolone and dexamethasone. These may be given on their own or, more commonly, along with chemotherapy or other anti-myeloma drugs. Corticosteroids are most often taken as tablets, but they can also be given intravenously.
Bisphosphonates are a class of drugs that reduce bone pain and prevent further bone weakness or damage caused by myeloma cells. They also prevent a dangerous rise in blood calcium levels and improve well-being. They work by coating the bone and blocking the activity of the cells that break down the bone. They may be given as a daily tablet or a monthly injection into a vein.
Bortezomib (Velcade®) belongs to a class of drugs that interfere with the growth of myeloma cells, causing them to stop growing and die. Bortezomib is injected intravenously, but it can also be injected under the skin (subcutaneously). Injections are generally given once or twice a week for the first two weeks followed by a 10 day break. It is often given with a steroid and sometimes chemotherapy.
The drug is available for people who have had initial treatment but the myeloma is still progressing.
Radiotherapy is a painless procedure that uses x-rays to kill myeloma cells or damage them so they cannot multiply. It is used to help relieve some symptoms such as bone pain.
Your radiation oncologist and haematologist will discuss the type of radiotherapy and the number of treatments you need. You will probably have treatment for a few days each week for several weeks.
Plasma exchange may be used if the level of paraprotein in the blood is very high and is interfering with blood circulation, making the blood thicker (hyperviscosity) and causing symptoms such as blurred vision and decreased alertness. Plasma exchange is a way of removing some of the extra proteins in the blood using a machine. A needle is placed into a vein in each arm. Blood is slowly removed from one arm and then passed through a separating machine. The healthy portion of the blood, plus new replacement plasma or albumin, is then put back in via the other arm. This process usually takes a few hours.
Stem cell transplant
A transplant of stem cells is an increasingly common treatment option for some myeloma patients. It enables you to receive high doses of chemotherapy that destroys the bone marrow and the myeloma cells. However, the transplanted blood-forming stem cells rescue the bone marrow and help restore blood cell numbers.
The goal of a transplant is to extend your life and improve your quality of life.
Most people receive their own previously collected stem cells (autologous transplant). These are usually collected from the bloodstream, but occasionally they are taken from the bone marrow. Sometimes stem cells from another person are used (allogeneic transplant).
A transplant is not suitable for everyone because of the risks associated with the high-dose chemotherapy given before the procedure (e.g. infection or excessive bleeding).
Palliative treatment helps improve quality of life by alleviating the symptoms of cancer without trying to cure the disease. It is particularly important for people with advanced cancer.
Often treatment is concerned with symptom control and stopping the spread of cancer, but it can also involve the management of other physical and emotional symptoms. Treatment may include radiotherapy, chemotherapy or other medication.
After your treatment is over, you will need regular checkups with your doctor or your cancer treatment centre. Blood tests will be done to check your general health and your blood count. Regular checkups can help find a recurrence early, and this gives you the best chance of getting the disease under control.
Checkups will continue for several years but will become less frequent if you have no further problems.
Between follow-up appointments, let your doctor know immediately of any health problems.
Understanding Multiple Myeloma
A guide for people with cancer, their families and friends.