Understanding Myeloma

About 20,580 Americans will be diagnosed with myeloma this year. It is estimated that approximately 66,529 people in the United States are living with or are in remission from myeloma.

Myeloma is a cancer of the plasma cells, a type of white cell found in many tissues of the body, but primarily in the bone marrow. Plasma cells are part of the body's immune system.

Normal plasma cells make antibodies, which  help fight infection. Myeloma cells cannot help the body fight infection. As the myeloma cells grow in the marrow they crowd out the normal plasma cells. They also crowd out normal white cells, red cells and platelets.

Most people with myeloma are age 50 or older. Americans of African descent are diagnosed with myeloma about twice as often as Americans of European descent. People of Asian and Hispanic descent have lower rates of myeloma than other groups.

This is a hopeful time for myeloma patients. Progress towards a cure is under way. New myeloma drugs have been approved in the last few years and new treatments are being studied.

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Causes

The exact cause of myeloma is not known.

Myeloma starts with a change to a single cell called a B lymphocyte. Normally, some B lymphocytes develop into plasma cells, which produce proteins called "antibodies" that can attach to an infectious agent and predispose it to be killed or removed.  With myeloma, the B lymphocyte becomes a myeloma cell instead of a normal plasma cell.

Researchers are studying myeloma to understand why this change occurs. There is no way to prevent myeloma. You cannot catch myeloma from someone who has it.

 

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Signs and Symptoms

In the early stage of myeloma some patients have no signs or symptoms. Often the first symptom of myeloma is bone pain caused by the effects of myeloma cells in the marrow. Most patients feel pain in their back or ribs, but it can occur in any bone.

Patients may have anemia, tire more easily and feel weak. Fractures may occur as a result of the weakened bones.

Patients may have recurrent infections because antibodies to invading viruses, bacteria or other disease agents are not made efficiently and not in adequate amounts.

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Finding a Myeloma Specialist

Myeloma patients are treated by oncologists or hematologists. It is best to choose a doctor who specializes and has experience in treating myeloma. Or have your local cancer specialist work with a myeloma specialist. Myeloma specialists know about new tests and treatments for your disease.

• Ask your primary care doctor
• Contact your community cancer center
• Call your local medical society
• Reach out to doctor and health plan referral services
• Call LLS for a list of cancer centers or visit www.LLS.org and click on "Cancer Centers"
• Use online physician-finder resources, such as The American Medical Association's "DoctorFinder" or the American Society of Hematology's "Find a Hematologist" 

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Types of Myeloma

Most myeloma patients (about 90 percent of cases) have disease that involves multiple sites in the body at the time of diagnosis. This is often called multiple myeloma.

A mass of myeloma cells is called a "plasmacytoma." A plasmacytoma can form in the bone, skin, muscle, lungs or any other part of the body. Some people have a single mass of myeloma cells outside the marrow, called an extramedullary plasmacytoma. Extramedullary means that the mass of myeloma cells is outside the marrow.

Rarely, people diagnosed with myeloma have solitary myeloma. This means they have one area of myeloma in the body.

Myeloma that progresses slowly is called smoldering or indolent myeloma. Sometimes patients with smoldering or indolent myeloma do not need treatment right away. In most cases, treatment is needed at some point.

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Diagnosis and Staging

Doctors do tests to look for signs of myeloma.

• Bone marrow aspiration and bone marrow biopsy
• Blood tests (including complete blood counts)
• Urine tests
• Imaging tests
  • X-rays (skeletal surveys)
  • CT scans
  • MRIs
  • PET scans

Bone marrow aspiration and biopsy may be done in the doctor's office or in a hospital. The marrow is looked at under a microscope to see if there are myeloma cells.

Lab tests are done to see if a protein called M protein or M spike is in the patient's blood and urine. The amount of M protein is one way to estimate the stage of the myeloma.

Another protein called light chains can be found in the myeloma patient's urine. This protein is also called Bence Jones protein.

Co-Pay Assistance Program support up to $10,000 for eligible Myeloma patients

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The whole immunoglobulin is composed of two larger pieces (heavy chains) and two smaller pieces (light chains) attached to each other. The Bence Jones protein (light chains) made by M protein are small enough to pass through the kidney and enter the urine, where they can be detected. When excreted in large amounts, Bence Jones protein can cause renal injury and kidney failure. There is a newer, special test to check for light chains. This test is called "serum-free light chains." 

Imaging tests include x-rays of areas of bone pain, x-rays of the skull, spine and ribs (skeletal survey), CT scans, MRIs and PET scans.

• X-rays and CT scans are used to see if there are any holes, breaks or thinning of the bones.
• MRIs and PET scans look for changes to the marrow and pockets of myeloma cells.

A test called FISH (fluorescence in situ hybridization) is used to see if there are changes to the chromosomes of the myeloma cells.

Lab and imaging tests are done to stage (measure the extent) of a patient's myeloma. Doctors determine the stage of the myeloma to help them decide which treatment approach to take.

For decades, a staging system called the "Durie-Salmon System" has been used. This system measures the patient's blood hemoglobin concentration, the amount of M protein in blood and urine, the level of blood calcium, the level of kidney function, and the presence of bone lesions on imaging studies. A newer and more accurate method, called the "International Staging System," relies on the degree of increase in the beta 2-microglobulin and the degree of decrease in serum albumin to determine disease stage.

To determine the stage of the disease, the doctor checks:

• Blood counts of red or white cells. These may be lower than normal with myeloma.
• The amount of M protein found in blood and urine. M protein is made by the myeloma cells.
• The calcium level in the blood. This may be higher than normal with myeloma.
• The beta 2-microglobulin level in the blood. This may be higher than normal with myeloma. Beta 2-microglobulin is a protein found on the surface of plasma cells and some other cells.
• The albumin (a protein in the blood) level, which may be lower than normal in myeloma patients.
• How many parts of the bones the myeloma has affected.

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Disease Complications

Myeloma patients may have problems with

• Infections. Myeloma patients may have more infections. This is because myeloma cells do not make antibodies to fight infection. Patients should follow the doctor's advice about how to reduce their risk. The doctor may give antibiotics to treat infections.
• Bone pain. Myeloma may cause bone pain. Drugs called bisphosphonates (Aredia® or Zometa®) may help. Bisphosphonates work by blocking the myeloma cells from making the bones weak.
• Kidney problems. Myeloma patients have a protein called light chains or Bence Jones protein. Myeloma patients may also have high levels of calcium in their blood. Each of these can damage the kidneys. The doctor will check the patient's kidneys.
• Acute myelogenous leukemia (AML). A small number of myeloma patients develop AML.

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Treatment

The goals of treatment for myeloma are to

• Slow the growth of the myeloma cells. 
• Help patients who have bone pain, fatigue or other problems from their disease to feel better.
• Provide long periods of remission (when there are no signs of myeloma and/or the myeloma is not causing health problems).

The treatment a patient receives depends on the type and stage of the myeloma and the patient's age and overall health. Most treatment plans include a combination of therapies. Some patients only need supportive care to reduce symptoms of anemia, high blood calcium levels, infections and/or bone damage. Others receive myeloma-specific therapies to treat disease progression and provide remissions.

Myeloma-specific therapies include

• Single or combination drug therapy.
• High-dose chemotherapy with one of three types of stem cell transplant
  • Autologous
  • Allogeneic (as part of clinical trials)
  • Reduced-intensity allogeneic
• Radiation therapy for local disease
• New and emerging drug therapies (as part of clinical trials)

Drug Therapy.  Drug therapy is the main treatment for myeloma. Before drug therapy begins, patients with symptomatic myeloma are assessed to determine if they are candidates for stem cell transplant. For transplant candidates, drug treatment begins with induction agents that do not cause bone marrow damage, for example, thalidomide (Thalomid®) and dexamethasone, bortezomib (Velcade®) and dexamethasone, or Velcade, pegylated liposomal doxorubicin (Doxil®) and dexamethasone. For patients who are not candidates for transplant, treatment may begin with a combination drug therapy, such as melphalan and prednisone, with Thalomid or Velcade. As many as six drugs are combined in some intensive treatment programs.

Drug therapy has led to sustained remissions in some patients. Temporary cessation or significant slowing of the disease may occur for a time. Achieving complete remission for long periods is being seen more often as newer, more effective drugs are developed.

Some Drugs Used To Treat Myeloma

• Bortezomib (Velcade®) is given by injection. It is approved by the FDA to treat people who have with myeloma (newly daignosed and previously treated patients). There are also a number of ongoing research studies (clinical trials) underway for Velcade. 
• Thalidomide (Thalomid®) is given by mouth. Thalomid is used with dexamethasone to treat newly diagnosed myeloma patients. It is also being studied together with other drugs.
• Lenalidomide (Revlimid®) is a drug like Thalomid. Some studies have indicated that in comparison to Thalomid, Revlimid may be safer and work better for certain myeloma patients. Revlimid is used with dexamethasone to treat myeloma patients who have already had at least one other type of treatment.
• Melphalan (Alkeran®) is a type of chemotherapy used to treat some myeloma patients. Melphalan may be combined with other drugs such as Velcade, Thalomid or Revlimid.

Stem Cell Transplantation. Autologous stem cell transplantation is an important therapy for many myeloma patients. This procedure uses the patient's own stem cells to restore blood cell production after intensive chemotherapy. Autologous transplant is associated with good response rates. It is relatively safe for many patients, including older patients. However, it is not appropriate for all patients and it is not a cure for myeloma. Patients should discuss the benefits and risks of transplantation with their physicians.

Allogeneic stem cell transplantation and reduced-intensity allogeneic stem cell transplantation are being studied in clinical trials for myeloma. See the Clinical Trials section for more information.

Radiation therapy. This treatment uses high-energy rays to kill myeloma cells. It is the main treatment for localized myeloma, such as solitary myeloma or plasmacytoma. Patients sometimes receive radiation therapy in preparation for stem cell transplantation. Carefully selected patients whose bone pain does not respond to chemotherapy may receive radiation therapy as well.  

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Clinical Trials

Doctors are testing new drugs and new combinations of drugs to treat myeloma. "Clinical trials" are used to study new drugs, new treatments or new uses for approved drugs or treatments. A growing number of clinical trials include older adults.

There are clinical trials for

• Newly diagnosed myeloma patients
• Patients who do not get a good response to treatment
• Patients who relapse after treatment
• Patients who continue treatment after remission (maintenance).

Some clinical trials test new ways to use drugs that are already approved. For example, changing the amount of the drug or giving the drug along with another type of treatment might be better. The more people who take part in clinical trials, the faster we will find better ways to treat myeloma. You can also call the Information Resource Center for information about clinical trials; or use our free clinical trials service.   

Stem Cell Transplants under Study in Clinical Trials. For myeloma patients, allogeneic stem cell transplants are used less often to treat myeloma than autologous stem cell transplants. With this type, stem cells from a donor are used. The donor can be a brother or sister. Or the donor can be another person with stem cells that "match" the patient's. It may be a good treatment for patients younger than age 55 who are not doing well with other treatments. For myeloma, allogeneic transplants are usually done as part of a clinical trial.

Doctors are working to make allogeneic stem cell transplants safer. A type of transplant called a "reduced-intensity transplant" is under study. A reduced-intensity transplant uses lower doses of chemotherapy than a standard allogeneic stem cell transplant. This treatment is also called a "nonmyeloablative" transplant. Older and sicker patients may be helped by this treatment.

Some patients may benefit from having two stem cell transplants. Studies are under way to see if it helps myeloma patients to have an autologous transplant and a reduced-intensity allogeneic stem cell transplant.

Ask your doctor if participating in a clinical trial is right for you. For more information about clinical trials, you can view, print or order the free LLS booklet Understanding Clinical Trials for Blood Cancers. You can also watch the free LLS Web video My Clinical Trials Journey.

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Side Effects of Treatment

Myeloma patients should talk with their doctors about side effects before they begin any type of treatment. Patients react to treatment in different ways. Sometimes there are very mild side effects. Other side effects may be serious and last a long time.

Some side effects of myeloma treatment may include

• Upset stomach and vomiting
• Mouth sores
• Constipation
• Extreme tiredness
• Infections
• Low red cell count (anemia)
• Low white cell count
• Low platelet count 
• Achy feeling
• Numb feeling in arms, hands, legs or feet.

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Response to Treatment

Your doctor does tests to see if treatment is working. The test results help the doctor to decide if changes to treatment are needed. Blood and urine tests are done to check blood cell counts, kidney function and growth of myeloma cells. A bone marrow biopsy is used to look at the amount and pattern of myeloma cells in the marrow. Imaging tests (x-rays, CT scans, MRIs and PET scans) are used to look at the bones and marrow. X-rays and CT scans are used to see if there are any holes, breaks or thinning of the bones. MRIs and PET scans look for changes to the marrow and for pockets of myeloma cells.

The doctor may use these terms to talk about a patient's response to treatment:

• Remission. No sign of disease 
• Complete remission or response. No sign of M protein in the blood and urine. Normal percentage of plasma cells or no sign of myeloma cells in marrow
• Partial remission or response. More than a 50 percent decrease in M protein in the blood.
• Complete molecular remission or response. No sign of myeloma cells in the marrow using very sensitive tests.
• Minimal response. Less than a 50 percent decrease in M protein in the blood.
• Progressive disease. At least a 25 percent increase in M protein in the blood, new areas of bone damage or a new mass of myeloma cells.

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Get More Information

The Leukemia & Lymphoma Society offers ongoing education programs featuring leading myeloma specialists discussing the latest issues in the diagnosis and treatment of myeloma. Visit the Myeloma Education Series page to find out about upcoming and archived programs. These programs are offered at no charge.

Further details on treatment and supportive care may be obtained from The Leukemia & Lymphoma Society's free booklets The Myeloma Guide. Information for Patients and Caregivers and Myeloma.

Visit the Myeloma Facts & Statistics page for more information on incidence and survival rates.

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Contact Us

The Leukemia & Lymphoma Society
Home Office
1311 Mamaroneck Ave.
White Plains, NY 10605

or call the Information Resource Center at (800) 955-4572