Myeloma

The information in this section about myeloma can help you to talk with members of your healthcare team and take an active role in your treatment. Knowing what to expect and being able to make informed decisions about your cancer treatment are important aspects of coping with your disease. You can skim sections to find what you want to read now and continue reading whenever you're ready for more information.

What You Should Know

The concept of multiple myeloma relates to the clinical presentation of disease at multiple sites. The term multiple is frequently omitted nowadays. The diagnosis of myeloma is made by a combination of (1) evidence of bone marrow infiltration by plasma cells, (2) a paraprotein in the circulation produced by the plasma cells and, (3) evidence of what is termed end-organ damage. This can consist of damage to the skeleton, bone marrow function or the kidneys. Occasionally, patients present with bone marrow involvement and a substantial detectable paraprotein but without evidence of end-organ damage. This is termed 'asymptomatic' or 'smoldering' myeloma. There is no immediate need for treatment in asymptomatic disease.

What You Should Do

Talk with your doctor about your tests and what the results mean. Talk with your doctor about all your treatment options and the results you can expect from treatment.

What Is Myeloma?

Myeloma is a type of cancer that begins in the bone marrow. It is a malignancy of plasma cells, the cells which normally makes antibodies to fight infection. (For other cancers which affect the blood cells, please see the section on leukemias.)

Blood & Bone Marrow

All the different types of blood cells are made in the bone marrow. This is a spongy material that fills the middle of some of the bigger bones. Blood cells all develop from “stem” cells. These stem cells mature into white cells, red cells and platelets. When they are fully-grown, they are released into the blood and circulate around the body to where they are needed.

Red blood cells carry oxygen around the body
White blood cells are essential for fighting infection
Platelets help the blood to clot

Plasma Cells

Plasma cells are a type of white blood cell. They are normally responsible for the production of antibodies and are found in the bone marrow. In myeloma, it is these plasma cells which are abnormal. They accumulate and interfere with the production of normal blood cells, and also weaken the bones, leading to fractures and elevated calcium levels. They are referred to as myeloma cells. Normally plasma cells make many types of proteins called antibodies or immunoglobulins, which respond to invading bacteria and antigens, thereby defending the body against infection.

Myeloma cells

Myeloma cells, on the other hand, do not wait for an invasion of antigens to produce proteins. Instead, they make large amounts of one abnormal protein called a paraprotein or M protein. This is a 'junk' protein which does not fight infection and has no real use. The myeloma cells can also interfere with the development of normal plasma cells.

This protein can also cause kidney problems and interfere with the production of normal antibodies leading to immunodeficiency.

The Main Types of Plasma cell disease

In each case of myeloma, there is only one type of antibody or immunoglobulin that is overproduced. In about 50% of cases, it is an IgG immunoglobulin. Approximately 20% of cases involve an IgA immunoglobulin. The remainder of cases involve overproduction of either kappa or lambda light chains (termed light chain myeloma) or, rarely, IgD or IgE.

There are a number of less common plasma cell diseases.

Extramedullary myeloma affects tissue other than bone marrow, such as skin, muscle or lung.

Another way for doctors to divide myeloma is into groups that describe how rapidly or slowly the disease is progressing:

Smoldering (indolent) myeloma NHL progresses slowly.
Asymptomatic myeloma has no symptoms even though the patient has the disease.
Symptomatic myeloma has related symptoms such as anemia, kidney damage and bone disease.

So unlike some of the other blood cancers myeloma does not break down in to clearly differentiated types. Your doctor will explain your condition to you.

The Causes of Myeloma

There is no obvious cause for myeloma. Long-term exposure to certain chemicals seems to increase the risk. For the majority of the cases, there is no obvious reason why the disease developed.

It is the second most common cancer of the blood. In Ireland, about 222 people are diagnosed each year with myeloma (figures for 2006). Generally people are over 60 when they are diagnosed, it is very rare in the age group under 40 with about 2% of cases occurring in this age-group.

Myeloma is more common in men than in women. Factors which have been tentatively lined with an increased risk include -

Exposure to chemicals- petrol, oil, benzene, pesticides, dioxins, paints, rubber, hair dyes
Occupation – agricultural workers, wood or leather wookers, hairdressers, painters and decorators
Exposure to viruses – HIV, hepatitis, herpes virus 8, simian virus 40
Exposure to radiation – atomic radiation, radiotherapy
Plasma cell condition – monoclonal gammopathy of uncertain significance (MUGS) and smoldering myeloma
Other conditions – autoimmune illness, pernicious anaemia, ankylosing spondylitis
Family history – if your parent sibling or child is diagnosed with myeloma, you are between 2 and 6 times more likely to develop it.

Current research is looking to see if weight and diet can be risk factors.

Symptoms of Myeloma

The symptoms of myeloma may be vague at first. But, as the condition develops, it can affect bones, the blood and the kidneys. About one in five patients have no symptoms.

Bone Effects

The most common symptom of myeloma is persistent abnormal bone pain or a fracture for no apparent reason. Pain is mostly in the back or the ribs, but can occur in any bone. The pain is usually made worse by movement.

The myeloma cells can destroy bone tissue. In most cases, soft spots or holes develop where the bone structure has been damaged. These are termed lytic lesions when seen on plain x-rays.

cytokines

Myeloma cells secrete various chemicals called cytokines that stimulate cells to dissolve bone. Myeloma cells secrete other chemicals that can interfere with the formation of new bones. Normally our bodies produce cells that dissolve bone (osteoclasts) and cells that help build new bone (osteoblasts). In myeloma, the osteoclasts work overtime, and the osteoblasts can't keep up, upsetting the cells' balance. This leads to holes in the bones, called lytic spots, and osteoporosis (low bone density). Bones can become frail enough to break or fracture in a minor fall or injury and even during normal activities, such as walking, lifting, sneezing or coughing. If not treated, the thin bones can cause long-lasting bone pain.

Blood Effects

The most common of these symptoms include

Fatigue
Weakness
Recurring infections
Abnormal bruising or bleeding

The growing number of abnormal plasma cells crowds out the space needed for other blood cells to reproduce. As a result, fewer red blood cells can lead to anaemia, and less oxygen being carried around the body. This results in fatigue and weakness. The effect of fewer white cells is that infections develop more easily. This includes repeated coughs, colds and flus (mainly chest infections). Because of the lower than normal number of platelets, unexplained bruising and bleeding, such as nose bleeds or bleeding gums, may occur. This is seldom seem, however, with newly diagnosed disease.

Kidney Effects

There may be increased thirst and thus an increased need to urinate. The kidneys make sure that the level of calcium in the body is kept at a safe level, if more calcium is being broken down, then the level of calcium in the bloodstream rises above the normal levels, causing hypercalcaemia. The kidneys get overworked, trying to get rid of this excess calcium. This can lead to an increased need to go to the toilet and dehydration as a result. Hypercalcaemia can also cause loss of appetite, nausea and vomiting, hazy vision, constipation, depression and drowsiness.

Other kidney problems may occur. The paraproteins released by the myeloma cells enter the kidneys and block what are called the collecting ducts. This can lead to kidney damage or kidney failure.

Diagnosis of Myeloma

Diagnosing myeloma usually involves performing a series of tests. Blood, urine, bone marrow and imaging tests are commonly used. In the early stages, there are usually no symptoms and myeloma isn't usually suspected until blood tests indicate an elevated globulin level. This may lead to more tests. If myeloma is suspected as a result, your doctor tests your blood again and will send you to a haematologist to help make the diagnosis. When your blood sample is sent to a lab for a full blood count (FBC), this can reveal whether myeloma cells are affecting normal blood cell development. A full physical exam will be done to check your general state of health and a full medical history will be taken.

Your doctor also checks your blood for:

Calcium levels. High levels can mean that calcium has moved out of your bones and into your bloodstream, putting your kidney health at risk.
Protein levels. By measuring certain proteins, your doctor can estimate the size and growth rate of myeloma tumours.
Urea nitrogen and creatinine levels. These proteins are measured to test your kidney function.

Your doctor tests your urine for Bence Jones protein, which can indicate the presence of myeloma. A kidney function test will check how well your kidney are working and for any signs of damage. In particular, the levels of urea, electrolytes and creatinine will be checked. Again, this is done through simple blood tests.

Because myeloma causes bones to thin or wear away, there will be a check kept on all the bones in the body. This may include imaging tests, such as x-rays, CT scans or MRI scans. Here, your doctor is looking for any evidence of bone damage.

A bone marrow test will be performed to look for myeloma cells in the bone marrow. A tiny sample of the bone marrow is taken and examined under a microscope. A sample of the liquid marrow is called an aspirate and a sample of bone is called a biopsy. Usually the sample is taken from the hip . This procedure is done in the hospital under a local anaesthetic. It takes about 15 minutes and does not require an over night stay. Normal bone marrow has less than 5% plasma cells, wheras in myeloma, it is between 10% and 90%. the bone marrow will also be checked for any abnormal chromosomes. This is called cytogenetic testing. About 7 out of 10 people with myeloma have chromosome changes. Chromosome 13 in particular can be abnormal.

Treatment of Myeloma

Myeloma is a highly individual disease. Very often it is slow moving but it can speed up. Because there is no standard treatment of myeloma, the treatment plans are individual. The main resource for your doctors is the battery of tests which will keep them informed as to how you are doing. These are the best tools for them to use.

Tests for myeloma fall into several groups:

Laboratory tests (blood and urine)
Imaging studies (skeleton)
Pathology studies (biopsies)
Genetic studies (done on biopsy specimens)

Notable celebrities who have had Myeloma

Dr. Mel Goldstein – Weather man
Geraldine Ferraro – Former Congresswomen and VP Candidate
Mickey Hargitay – Hungarian Mr Universe
Sam Walton – Co founder of Wal-Mart
Louis Rukeyser – American TV host
Don Baylor – Is a major league Baseball coach
Ann Landers- Was the pen name created by the Chicago Times in 1943, Esther Lederer wrote the column between 1955 and 2002
Roy Scheider – Actor – the police chief in Jaws, he also acted in ‘All that Jazz’, ‘the French Connection and in the TV scfi series ‘SeaQuest DSV’
General Wayne Downing
Peter Boyle of “Everybody loves Raymond”