What it is, How its Treated and How to Cope

The body’s immune system is made up of (among other things) cells called plasma cells. When these plasma cells become malignant or cancerous we develop what is called plasmacytomas (a single tumor) or multiple myeloma (widespread tumors). Myeloma is, therefore, a form of cancer that occurs when plasma cells grow too quickly and in an abnormal way.

How the Immune System is Implicated in Myeloma

The lymphocytes or lymph cells in our immune systems are vital to its infection fighting ability and there are there are two types of lymphocytes namely: T cells and B cells, also called T and B ‘lymphocytes’.

B lymphocytes respond to an infection by maturing and forming into plasma cells which are housed mainly in the bone marrow which is the soft, inner part of our bones. These cells make and release a kind of protein called ‘antibodies’ or immunoglobulins which seek out and destroy bacteria and other germs that enter our bodies.

These cells can develop into a tumor if they begin to grow in an uncontrolled way and often start in the body’s bone marrow. Single tumors are called plasmacytomas but in most cases the tumors are widespread in the bone marrow and this is called multiple myeloma. Even though they produce antibodies the abnormal cells do not help to protect the body against infections.

When the plasma cells begin to grow too rapidly and in an abnormal way they damage the normal blood-forming functions carried out by the bone marrow and cause too few red blood cells to be produced. This is known as anemia and can make you feel very tired or breathless. Overgrowth of plasma cells also cause too few blood platelets to be produced and since these cells seal damaged blood vessels excessive bleeding can occur if you injure yourself.

If there are too many plasma cells in your bone marrow you may develop leucopenia where there are too few white blood cells and this may affect your body’s resistance to infection.

What are Solitary Plasmacytomas?

Solitary plasmacytomas are single tumors in the bone marrow or in other tissues like the lungs or the lining of the sinuses, throat (known as ‘extramedullary disease’). Most patients with this kind of plasmacytoma go on to develop multiple myeloma and this is especially so is the original tumor was found in areas of bone. If the tumors do not develop into the multiple form of the disease, most patients can be treated successfully.

What is Multiple Myeloma and How Does it Develop in the Body?

Multiple myeloma is sometimes preceded by ‘monoclonal gammopathy of undetermined significance’ or MGUS which is also a condition in which the plasma cells of the body grow abnormally and too rapidly. MGUS is often found in elderly people.

This illness causes damaged plasma cells to give off antibody proteins in large amounts but they don’t form a tumor or mass and there are no symptoms or ill effects on health. Sometimes MGUS is found during a routine blood test when high protein levels in the blood are found to be immunoglobulins.

Patients that are diagnosed with MGUS often go on to develop multiple myeloma, lymphoma, or amyloidosis at about 1 percent a year. If the protein levels are very high the risk is increased and people who have MGUS need to be monitored carefully for signs of multiple myeloma even though they don’t need to be treated with drugs at this stage.

The genetic make-up of the plasma cells of those with MGUS are found to be inherently malignant, but not growing rapidly at that point in time. Often patients may die of natural causes before multiple myelomas ever develop.

What are the Risk Factors for Myeloma?

Having a risk factor for a specific disease does not necessarily mean that you are sure to get it but it may increase your chances of developing the illness.

Studies carried out on people with myeloma have revealed certain factors that may increase your risk of getting the disease.

• Your age may increase your chances of getting myeloma as might your particular gender. People who are diagnosed with the illness have an average age of sixty eight years old and more men get it than do women.

• Your race may also affect your risk of getting myeloma – twice as many people who are African American get it than do Caucasian people or other race groups. It is not understood why this is so.

• If you have been exposed to radiation in any form, including that to cure other forms of cancer, you might have a higher risk of developing myeloma but the risk is very small.

• Some people have several relatives with the disease but this is very rare and most people seem to have no genetically inherited risk.

• People who work in petroleum-related industries may show an increased risk.

• Obese people have been found to have a higher incidence of myeloma in some studies.

• If you have MGUS or solitary plasmacytoma you will probably develop multiple myeloma at some stage.

What are the Symptoms of Multiple Myeloma?

It is not always possible to diagnose multiple myeloma at an early stage of the disease and it probably won’t cause any symptoms early on. In some patients there may be vague symptoms but these are likely to be misdiagnosed. As we have already mentioned it may be discovered during routine blood tests that reveal excessive amounts of proteins in the blood.

If symptoms do occur they may include some or all of the following:

• Bone pain may occur due to the plasma cell tumors releasing substances that activate and block bone-producing functions causing the bone to become weak and painful or to break. Pain in the backbone, hip bones and skull is a common symptom of the disease.

• Your blood may show anomalies like anemia due to decreased levels of red blood cells or a large number of infections may occur because your white blood cells are decreased leading to a condition called leukopenia. If you have too few platelets in your blood you may develop thrombocytopenia and experience excessive bleeding even with minor bruises or cuts.

• Your nervous system may be affected causing weakness and collapse of spinal bones which in turn causes nerve compression with pain, numbness and muscle weakness. If you experience any of these symptoms it is a medical emergency and you should contact your doctor immediately. Rising levels of calcium in the blood may cause mental confusion.

• Excessive amounts of protein in the blood may cause nerve damage with weakness and numbness. The blood flow to the brain may be obstructed and cause stroke-like symptoms, mental confusion or dizziness. Call your doctor immediately if this occurs as the condition can be reversed by a procedure that removes the protein from the blood.

• High protein levels can also damage the kidneys and your body may not be able to get rid of excess salt, fluid, and waste products. Kidney failure usually causes weakness and leg swelling.

• High blood calcium levels may cause the bones to dissolve rapidly so that excess calcium is released into the bloodstream. This is called hypercalcemia and may cause kidney failure. If you feel very thirsty and ‘dry’ as well as have appetite loss, constipation or a drowsy, sleepy feeling call your doctor.

• You may get more infections. Myeloma patients are about 15 times more likely to have lowered infection resistance. As a result you may develop pneumonia or other opportunistic diseases.

How is Multiple Myeloma Treated?

The way in which myeloma is treated has undergone some dramatic changes in recent years due to the introduction of blood-forming stem cell transplantation. Ask your doctor about blood-forming stem cell transplantation and if this therapy is suitable to treat your illness.

Before this therapy was introduced, two chemotherapy drugs, namely melphalan and prednisone were used as well as vincristine, cyclophosphamide, carmustine, and doxorubicin combined with the two major drugs. Drugs are given in combination to increase their effectiveness in the body and are used when the disease is widespread.

Chemotherapy may be given by mouth or injected into a vein or muscle where it quickly goes into the bloodstream and attacks the cancer. The drugs you will be given depend on what stage of cancer you have, how old you are and how well your kidneys are functioning. If you are also going to have a stem cell transplantation you will not be given melphalan, cyclophosphamide or carmustine as these can damage the bone.

Radiation therapy may be used if chemotherapy is not effective. Radiation treatments use high energy x-rays to penetrate the body tissues and kill the cancer. External beam radiation is used on patients who have myeloma. The radiation is given from outside the body using a machine.

Thalidomide is sometimes used to treat myeloma. When given with dexamethasone seventy percent of patients have a partial or complete disappearance of their illness and before blood-forming stem cell transplantation it was widely used. It has major side effects like severe constipation, numbness and tingling of extremities, and fatigue and sleepiness.

Bortezomib (Velcade) or PS-341 has been approved by the FDA if the patient does not improve after at least two other forms of treatment. This drug is a proteasome inhibitor and works to prevent enzyme complexes or ‘proteasomes’ within cells from breaking down the proteins that keep cell division under control. This drug does have several side effects.

What are the Side Effects of Chemotherapy?

Chemotherapy drugs are effective in killing cancer cells but can also damage normal, healthy cells in the body, in particular those that are rapidly growing, so you may experience some side effects from your treatments.

You may not get all or even any of these side effects and they do depend largely on the kind of drugs you are given and how much and how long you take them for. The good news is that most of these effects disappear as soon as chemotherapy is stopped and cells start to re-grow.

Your bone marrow, mouth lining and intestines, and hair follicles may be the first to be affected and you may develop:

• Hair loss

• Mouth sores

• Compromised resistance to infection due to low white blood cell counts

• Bruising or bleeding due to low blood platelets

• Fatigue due to low red blood cells

• Loss of appetite

• Nausea, and vomiting from damage to intestinal cells

Can Side Effects be Lessened or Avoided?

Your doctor can give you drugs to prevent or reduce nausea and vomiting along with your chemotherapy medicine. The drugs you are given may also cause more serious effects on the heart or kidneys and if so, they will be discontinued and another medication given. Ask your doctor about ways your side effects may be reduced if you are in a lot of discomfort.

To conclude, make sure you know as much about your illness as possible and ask questions of your doctor and care team about the goals of treatment, the medicines you will be given and the side effects you may experience.

Pay attention to your own needs at this time. A positive and balanced outlook will keep you strong and help your treatments to work better. Make sure you get enough, rest, moderate exercise and follow a healthy, balanced diet. Talk over any stressful feelings with a concerned listener or group. Positive lifestyle strategies can go a long way in helping you to survive cancer.