Multiple Myeloma
Isolated Plasmacytoma of Bone
Extramedullary Plasmacytoma
Waldenström Macroglobulinemia (Lymphoplasmacytic Lymphoma)
Monoclonal Gammopathy of Undetermined Significance
Refractory Plasma Cell Neoplasms

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Patients without symptoms may not need treatment. When symptoms appear, the treatment of multiple myeloma may be done in phases:

Induction therapy: This is the first phase of treatment. Its purpose is to reduce the amount of disease, and may include one or more of the following:

• Corticosteroid therapy.
• Thalidomide or lenalidomide therapy.
• Bortezomib therapy.
• Chemotherapy.

Clinical trials of different combinations of treatment should be considered.

Consolidation chemotherapy: This is a type of high-dose chemotherapy often given as the second phase of treatment, and may include either:

• autologous stem cell transplant, in which the patient's own stem cells are used; or
• allogeneic stem cell transplant, in which the patient receives stem cells from a donor.

Maintenance therapy: After the initial treatment, maintenance therapy is often given to help keep the disease in remission for a longer time. Several types of treatment are being studied for this use, including:

• Chemotherapy.
• Biologic therapy.
• Corticosteroid therapy.
• Thalidomide therapy.

Supportive care to treat bone problems and amyloidosis may include:

• Bisphosphonate therapy to slow bone loss and reduce bone pain. See the following PDQ summaries for more information about bisphosphonates and problems related to their use:
  • Pain ¹
  • Oral Complications of Chemotherapy and Head/Neck Radiation ²
• Radiation therapy for tumors of the spine.
• Chemotherapy to reduce back pain from osteoporosis or compression fractures of the spine.
• Chemotherapy and corticosteroid therapy to treat amyloidosis.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with multiple myeloma ³. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Standard treatment of isolated plasmacytoma of bone is usually radiation therapy.

Supportive care to treat amyloidosis may include chemotherapy and corticosteroid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with isolated plasmacytoma of bone ⁵. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Standard treatment of extramedullary plasmacytoma may include the following:

• Radiation therapy to the tumor and nearby lymph nodes.
• Surgery, usually followed by radiation therapy.
• Watchful waiting after initial treatment, followed by radiation therapy, surgery, or chemotherapy if the tumor grows or causes symptoms.

Supportive care to treat amyloidosis may include chemotherapy and corticosteroid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with extramedullary plasmacytoma ⁶. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Treatment of Waldenström macroglobulinemia may include the following:

• Plasmapheresis and chemotherapy.
• Chemotherapy with one or more drugs.
• Watchful waiting.
• Biologic therapy with monoclonal antibodies.
• A clinical trial of stem cell transplant.

Supportive care to treat amyloidosis may include chemotherapy and corticosteroid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with Waldenstrom macroglobulinemia ⁷. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Treatment of monoclonal gammopathy of undetermined significance (MGUS) is usually watchful waiting, which will include regular blood tests to check the level of M protein in the blood.

Supportive care to treat amyloidosis may include chemotherapy and corticosteroid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with monoclonal gammopathy of undetermined significance ⁸. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Treatment of refractory plasma cell neoplasms may include the following:

• Watchful waiting for patients whose disease is stable.
• A different treatment than previously given. (See Multiple Myeloma ⁹ treatment options.)

Supportive care to treat amyloidosis may include chemotherapy and corticosteroid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with refractory multiple myeloma ¹⁰. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site ⁴.

Glossary Terms

A procedure in which a person receives blood-forming stem cells (cells from which all blood cells develop) from a genetically similar, but not identical, donor. This is often a sister or brother, but could be an unrelated donor.

A group of diseases in which protein is deposited in specific organs (localized amyloidosis) or throughout the body (systemic amyloidosis). Amyloidosis may be either primary (with no known cause) or secondary (caused by another disease, including some types of cancer). Generally, primary amyloidosis affects the nerves, skin, tongue, joints, heart, and liver; secondary amyloidosis often affects the spleen, kidneys, liver, and adrenal glands.

A procedure in which blood-forming stem cells (cells from which all blood cells develop) are removed, stored, and later given back to the same person.

Treatment to boost or restore the ability of the immune system to fight cancer, infections, and other diseases. Also used to lessen certain side effects that may be caused by some cancer treatments. Agents used in biological therapy include monoclonal antibodies, growth factors, and vaccines. These agents may also have a direct antitumor effect. Also called biological response modifier therapy, biotherapy, BRM therapy, and immunotherapy.

A drug or substance used to treat hypercalcemia (abnormally high blood calcium) and bone pain caused by some types of cancer. Forms of bisphosphonates are also used to treat osteoporosis and for bone imaging. Bisphosphonates inhibit a type of bone cell that breaks down bone. Also called diphosphonate.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

A drug used to treat multiple myeloma that has gotten worse during treatment with other anticancer drugs. It is also used to treat mantle cell lymphoma in patients who have already received at least one other type of treatment. Bortezomib is also being studied in the treatment of other types of cancer. It blocks several cell functions and may cause cancer cells to die. It is a type of proteasome inhibitor and a type of dipeptidyl boronic acid. Also called PS-341 and Velcade.

Treatment with drugs that kill cancer cells.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.

Treatment that is given after cancer has disappeared following the initial therapy. Consolidation therapy is used to kill any cancer cells that may be left in the body. It may include radiation therapy, a stem cell transplant, or treatment with drugs that kill cancer cells. Also called intensification therapy and postremission therapy.

Any steroid hormone made in the adrenal cortex (the outer part of the adrenal gland). They are also made in the laboratory. Corticosteroids have many different effects in the body, and are used to treat many different conditions. They may be used as hormone replacement, to suppress the immune system, and to treat some side effects of cancer and its treatment. Corticosteroids are also used to treat certain lymphomas and lymphoid leukemias.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

An intensive drug treatment to kill cancer cells, but that also destroys the bone marrow and can cause other severe side effects. High-dose chemotherapy is usually followed by bone marrow or stem cell transplantation to rebuild the bone marrow.

Initial treatment used to reduce a cancer. Induction therapy is followed by other treatments, such as chemotherapy, radiation therapy, and hormone therapy to get rid of cancer that remains. Also called first-line therapy, primary therapy, and primary treatment.

A drug that is similar to thalidomide, and is used to treat multiple myeloma and certain types of anemia. It is also being studied in the treatment of other types of cancer. Lenalidomide belongs to the family of drugs called angiogenesis inhibitors. Also called CC-5013 and Revlimid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

An antibody found in unusually large amounts in the blood or urine of people with multiple myeloma and other types of plasma cell tumors. Also called monoclonal protein.

Treatment that is given to help keep cancer from coming back after it has disappeared following the initial therapy. It may include treatment with drugs, vaccines, or antibodies that kill cancer cells, and it may be given for a long time.

A type of protein made in the laboratory that can locate and bind to substances in the body, including tumor cells. There are many kinds of monoclonal antibodies. Each monoclonal antibody is made to find one substance. Monoclonal antibodies are being used to treat some types of cancer and are being studied in the treatment of other types. They can be used alone or to carry drugs, toxins, or radioactive materials directly to a tumor.

A benign condition in which there is a higher-than-normal level of a protein called M protein in the blood. Patients with monoclonal gammopathy of undetermined significance are at an increased risk of developing cancer. Also called MGUS.

A type of cancer that begins in plasma cells (white blood cells that produce antibodies). Also called Kahler disease, myelomatosis, and plasma cell myeloma.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Neoplasms may be benign (not cancer), or malignant (cancer). Also called tumor.

A condition that is marked by a decrease in bone mass and density, causing bones to become fragile.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

A type of immune cell that makes large amounts of a specific antibody. Plasma cells develop from B cells that have been activated. A plasma cell is a type of white blood cell. Also called plasmacyte.

A type of cancer that begins in plasma cells (white blood cells that produce antibodies). A plasmacytoma may turn into multiple myeloma.

The process of separating certain cells from the plasma in the blood by a machine; only the cells are returned to the person. Plasmapheresis can be used to remove excess antibodies from the blood.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

In medicine, describes a disease or condition that does not respond to treatment.

A decrease in or disappearance of signs and symptoms of cancer. In partial remission, some, but not all, signs and symptoms of cancer have disappeared. In complete remission, all signs and symptoms of cancer have disappeared, although cancer still may be in the body.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spine encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spinal column, and vertebral column.

Cancer that is neither decreasing nor increasing in extent or severity.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called best practice and standard of care.

A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.

A method of replacing immature blood-forming cells that were destroyed by cancer treatment. The stem cells are given to the person after treatment to help the bone marrow recover and continue producing healthy blood cells.

Care given to improve the quality of life of patients who have a serious or life-threatening disease. The goal of supportive care is to prevent or treat as early as possible the symptoms of a disease, side effects caused by treatment of a disease, and psychological, social, and spiritual problems related to a disease or its treatment. Also called comfort care, palliative care, and symptom management.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A drug that is used to treat multiple myeloma in patients who have just been diagnosed, and a painful skin disease related to leprosy. It is also being studied in the treatment of other types of cancer. Thalidomide belongs to the family of drugs called angiogenesis inhibitors. Also called Thalomid.

Treatment.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

An indolent (slow-growing) type of non-Hodgkin lymphoma marked by abnormal levels of IgM antibodies in the blood and an enlarged liver, spleen, or lymph nodes. Also called lymphoplasmacytic lymphoma.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called active surveillance, expectant management, and observation.