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Table of Contents General Information About Multiple Myeloma and Other Plasma Cell Neoplasms
Stages of Multiple Myeloma and Other Plasma Cell Neoplasms
Refractory Multiple Myeloma and Other Plasma Cell Neoplasms
Treatment Option Overview
Treatment Options for Multiple Myeloma and Other Plasma Cell Neoplasms
To Learn More About Multiple Myeloma and Other Plasma Cell Neoplasms
Get More Information From NCI
Changes to This Summary (07/24/2008)
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General Information About Multiple Myeloma and Other Plasma Cell Neoplasms
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Multiple myeloma and other plasma cell neoplasms (cancers) are diseases in which the body makes too many plasma cells.
Plasma cells develop from B lymphocytes (B cells), a type of white blood cell that is made in the bone marrow. Normally, when bacteria or viruses enter the body, some of the B cells will change into plasma cells. The plasma cells make a different antibody to fight each type of bacteria or virus that enters the body, to stop infection and disease. | Enlarge | | | | Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell. |
Plasma cell neoplasms are diseases in which there are too many plasma cells, or myeloma cells, that are unable to do their usual work in the bone marrow. When this happens there is less room for healthy red blood cells, white blood cells, and platelets. This condition may cause anemia or easy bleeding, or make it easier to get an infection. The abnormal plasma cells often form tumors in bones or soft tissues of the body. The plasma cells also make an antibody protein, called M protein, that is not needed by the body and does not help fight infection. These antibody proteins build up in the bone marrow and can cause the blood to thicken or can damage the kidneys.
There are several types of plasma cell neoplasms.
Plasma cell neoplasms include the following:
Multiple myeloma
In multiple myeloma, abnormal plasma cells (myeloma cells) build up in the bone marrow, forming tumors in many bones of the body. These tumors may prevent the bone marrow from making enough healthy blood cells. Normally, the bone marrow produces stem cells (immature cells) that develop into three types of mature blood cells:
As the number of myeloma cells increases, fewer red blood cells, white blood cells, and platelets are made. The myeloma cells also damage and weaken the hard parts of the bones. Sometimes multiple myeloma does not cause any symptoms. The following symptoms may be caused by multiple myeloma or other conditions. A doctor should be consulted if any of the following problems occur:
- Bone pain, often in the back or ribs.
- Bones that break easily.
- Fever for no known reason or frequent infections.
- Easy bruising or bleeding.
- Trouble breathing.
- Weakness of the arms or legs.
- Feeling very tired.
A tumor can damage the bone and cause hypercalcemia (a condition in which there is too much calcium in the blood). This can affect many organs in the body, including the kidneys, nerves, heart, muscles, and digestive tract, and cause serious health problems.
Hypercalcemia may cause the following symptoms:
Plasmacytoma
In this type of plasma cell neoplasm, the abnormal plasma cells (myeloma cells) collect in one location and form a single tumor, called a plasmacytoma. A plasmacytoma may form in bone marrow or may be extramedullary (in soft tissues outside of the bone marrow). Plasmacytoma of the bone often becomes multiple myeloma. Extramedullary plasmacytomas commonly form in tissues of the throat and sinuses; these usually can be cured.
Symptoms depend on where the tumor is.
- In bone, the plasmacytoma may cause pain or broken bones.
- In soft tissue, the tumor may press on nearby areas, causing pain or other problems. A plasmacytoma in the throat, for example, can make it difficult to swallow.
Macroglobulinemia
In macroglobulinemia, abnormal plasma cells build up in the bone marrow, lymph nodes, and spleen. They make too much M protein, which causes the blood to become thick. The lymph nodes, liver, and spleen may become swollen.
The thickened blood may cause problems with blood flow in small blood vessels.
Symptoms of macroglobulinemia depend on the part of the body affected. Most patients with macroglobulinemia have no symptoms. A doctor should be consulted if any of the following problems occur:
- Feeling very tired.
- Headache.
- Nosebleeds.
- Vision changes such as blurred vision or bulging eyes.
- Dizziness.
- Pain, tingling, or numbness in the hands, feet, fingers, toes, or other parts of the body.
- Trouble walking.
- Confusion.
- Pain or a feeling of fullness below the ribs on the left side.
- Painless lumps in the neck, underarm, stomach, or groin.
Monoclonal gammopathy of undetermined significance (MGUS)
In this type of plasma cell neoplasm, there are abnormal plasma cells in the bone marrow but there is no cancer. The abnormal plasma cells produce M protein that may be found during a routine blood or urine test. In most patients, the amount of M protein stays the same and there are no symptoms or problems. In some patients, MGUS may later become a more serious condition, such as multiple myeloma or lymphoma.
Multiple myeloma and other plasma cell neoplasms may cause a condition called amyloidosis.
In rare cases, multiple myeloma can cause organs to fail. This may be caused by a condition called amyloidosis. Antibody proteins build up and may bind together and collect in organs, such as the kidney and heart. This can cause the organs to become stiff and unable to work the way they should.
Age can affect the risk of developing plasma cell neoplasms.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor.
Plasma cell neoplasms are found most often in people who are middle aged or older. For multiple myeloma and plasmacytoma, other risk factors include the following:
- Being black.
- Being male.
- Having a brother or sister who has multiple myeloma.
- Being exposed to atomic bomb radiation.
Tests that examine the blood, bone marrow, and urine are used to detect (find) and diagnose multiple myeloma and other plasma cell neoplasms.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Biopsy: The removal of bone cells, lymph nodes, or tissues so they can be viewed under a microscope by a pathologist to check for abnormal cells or signs of cancer.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells.
| Enlarge | | | | Bone marrow aspiration and biopsy. After a small area of skin is numbed, a Jamshidi needle (a long, hollow needle) is inserted into the patient’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope. |
- X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body. The x-rays are used to find areas where the bone is damaged.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI). An MRI may be used to find areas where the bone is damaged.
- Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells and platelets.
- The number and type of white blood cells.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells.
- Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as calcium or albumin, released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
- Blood or urine immunoglobulin studies: A procedure in which a blood or urine sample is checked to measure the amounts of certain antibodies (immunoglobulins). For multiple myeloma, beta-2-microglobulin, M protein, and other proteins made by the myeloma cells are measured. A higher-than-normal amount of these substances can be a sign of disease.
- Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of certain substances. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. A higher than normal amount of protein may be a sign of multiple myeloma.
- Electrophoresis: A test in which a blood or urine sample is checked for M proteins and the amount of M proteins is measured.
- Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) depends on the following:
- The type of plasma cell neoplasm.
- The stage of the disease.
- Whether a certain immunoglobulin (antibody) is present.
- Whether there are certain genetic changes.
- Whether the kidney is damaged.
- Whether the cancer responds to initial treatment or recurs (comes back).
Treatment options depend on the following:
- The type of plasma cell neoplasm.
- The age and general health of the patient.
- Whether there are health problems related to the disease.
- Whether the cancer responds to initial treatment or recurs (comes back).
 Back to Top Stages of Multiple Myeloma and Other Plasma Cell Neoplasms
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After multiple myeloma and other plasma cell neoplasms have been diagnosed, tests are done to find out the amount of cancer in the body.
The process used to find out the amount of cancer in the body is called staging. It is important to know
the stage in order to plan treatment. The following tests and
procedures may be used in the staging process:
- X-ray: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the bone marrow. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radionuclide glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
- Bone densitometry: A procedure that uses a special type of x-ray to measure bone loss.
Certain tests may be repeated to see how well the treatment is working.
The stage of multiple myeloma is based on the levels of beta-2-microglobulin and albumin in the blood.
Beta-2-microglobulin and albumin are found in the blood. Beta-2-microglobulin is a protein found on the surface of plasma cells. Albumin makes up the biggest part of the blood plasma. It keeps fluid from leaking out of blood vessels, brings nutrients to tissues, and carries hormones, vitamins, drugs, and other substances, such as calcium, throughout the body. The amount of beta-2-microglobulin is increased and the amount of albumin is decreased in the blood of patients with multiple myeloma.
The following stages are used for multiple myeloma:
Stage I multiple myeloma
In stage I multiple myeloma, the blood levels are as follows:
Stage II multiple myeloma
In stage II multiple myeloma, the blood levels are as follows:
- beta-2-microglobulin level is lower than 3.5 g/mL and the albumin level is lower than 3.5 g/dL; or
- beta-2-microglobulin level is as high as 3.5 g/mL but lower than 5.5 g/mL.
Stage III multiple myeloma
In stage III multiple myeloma, the blood level of beta-2-microglobulin is 5.5 g/mL or higher.
The stages of other plasma cell neoplasms are different from the stages of multiple myeloma.
Isolated plasmacytoma of bone
In isolated plasmacytoma of bone, one plasma cell tumor is found in the bone, less than 5% of the bone marrow is made up of plasma cells, and there are no other signs of cancer.
Extramedullary plasmacytoma
One plasma cell tumor is found in the soft tissue but not in the bone or the bone marrow.
Macroglobulinemia
There is no standard staging system for macroglobulinemia.
Monoclonal Gammopathy of Undetermined Significance
In monoclonal gammopathy of undetermined significance (MGUS), less than 10% of the bone marrow is made up of plasma cells, there is M protein in the blood, and there are no other signs of cancer.
Back to Top Refractory Multiple Myeloma and Other Plasma Cell Neoplasms
Multiple myeloma and other plasma cell neoplasms are called refractory when the number of plasma cells continues to increase even though treatment is given.
Back to Top Treatment Option Overview
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There are different types of treatment for patients with multiple myeloma and other plasma cell neoplasms.
Different types of treatments are available for patients with multiple myeloma and other plasma cell neoplasms. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Nine types of standard treatment are used:
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Other drug therapy
Corticosteroid therapy
Corticosteroids are steroids that have antitumor effects in lymphomas and lymphoid leukemias.
Thalidomide and lenalidomide
Thalidomide and lenalidomide are drugs called angiogenesis inhibitors that prevent the growth of new blood vessels into a solid tumor.
Bortezomib
Bortezomib is a type of drug called a proteasome inhibitor that targets certain proteins in cancer cells and may prevent the growth of tumors.
High-dose chemotherapy with stem cell transplant
This treatment is a way of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Biologic therapy
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Monoclonal antibody therapy is one type of biologic therapy. It is a cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.
Surgery
Surgery to remove the tumor may be done, usually followed by radiation therapy. Treatment given after the surgery, to increase the chances of a cure, is called adjuvant therapy.
Watchful waiting
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
Plasmapheresis
Plasmapheresis is a procedure in which blood is removed from the patient and sent through a machine that separates the plasma (the liquid part of the blood) from the blood cells. The patient's plasma contains the unneeded antibodies and is not returned to the patient. The normal blood cells are returned to the bloodstream along with donated plasma or a plasma replacement. Plasmapheresis does not prevent new antibodies from forming.
Supportive care
This therapy controls problems or side effects caused by the disease or its treatment, and improves quality of life. Supportive care is given to treat bone problems or amyloidosis related to multiple myeloma and other plasma cell neoplasms.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site.
New combinations of therapies
Clinical trials are studying different combinations of biologic therapy, chemotherapy, steroid therapy, and drugs such as thalidomide or lenalidomide.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has | 
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