Multiple Myeloma and Other Plasma Cell Neoplasms

Key Points for This Section

  • Multiple myeloma and other plasma cell neoplasms (cancers) are diseases in which the body makes too many plasma cells.
  • There are several types of plasma cell neoplasms.
    • o Multiple myeloma
    o Plasmacytoma
    o Macroglobulinemia
    o Monoclonal gammopathy of undetermined significance (MGUS)
  • Age can affect the risk of developing plasma cell neoplasms.
  • Tests that examine the blood, bone marrow, and urine are used to detect (find) and diagnose multiple myeloma and other plasma cell neoplasms.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

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.

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 tumours 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 tumours in many bones of the body. These tumours 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:

  • Red blood cells that carry oxygen and other materials to all tissues of the body.
  • White blood cells that fight infection and disease.
  • Platelets that help prevent bleeding by causing blood clots to form.

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 tumour 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:

  • Loss of appetite.
  • Nausea or vomiting.
  • Feeling thirsty.
  • Frequent urination.
  • Constipation.
  • Feeling very tired.
  • Muscle weakness.
  • Restlessness.
  • Mental confusion or trouble thinking.

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 function.

Plasmacytoma

In this type of plasma cell neoplasm, the abnormal plasma cells (myeloma cells) collect in one location and form a single tumour, 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 tumour is.

  • In bone, the plasmacytoma may cause pain or broken bones.
  • In soft tissue, the tumour 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.

Age can affect the risk of developing plasma cell neoplasms.

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 to check for abnormal cells or signs of cancer.
  • Bone marrow aspiration and biopsy: The removal of a small piece of bone and bone marrow by inserting a needle into the hipbone or breastbone. A pathologist views both the bone and the bone marrow samples under a microscope to look for abnormal cells.
  • X-rays: 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.
  • Complete blood count (CBC) with differential: A procedure in which a sample of blood is drawn and checked for the following:
    • o The number of red blood cells and platelets.
    o The number and type of white blood cells.
    o The amount of haemoglobin (the protein that carries oxygen) in the red blood cells.
    o 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, 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 produces it.
  • Blood immunoglobulin studies: A procedure in which a blood sample is checked to measure the amounts of antibodies (immunoglobulins) and other proteins made by the myeloma cells. 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 produces 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 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).

Stages of Multiple Myeloma and Other Plasma Cell Neoplasms

Key Points for This Section

  • 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 stage of multiple myeloma is based on the number of myeloma cells in the body.
  • The following stages are used for multiple myeloma:
    • o Stage I multiple myeloma
    o Stage II multiple myeloma
    o Stage III multiple myeloma
  • Other plasma cell neoplasms are staged differently than multiple myeloma.
    • o Isolated plasmacytoma of bone
    o Extramedullary plasmacytoma
    o Macroglobulinemia
    o Monoclonal Gammopathy of Undetermined Significance

    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-rays: 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, computerised tomography, or computerised axial tomography.
    • PET scan (positron emission tomography scan): A procedure to find malignant tumour 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 tumour cells show up brighter in the picture because they are more active and take up more glucose than normal cells.
    • 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 number of myeloma cells in the body.

    There are three stages for multiple myeloma. The number of myeloma cells in the body is determined by the following:

    • Level of haemoglobin in the blood.
    • Levels of calcium and creatinine in the blood.
    • Amount of bone damage.
    • Amount of antibody M protein in the blood and/or urine.

    It is also important to know how well the kidneys are working.

    The following stages are used for multiple myeloma:

    Stage I multiple myeloma

    In stage I multiple myeloma, there is a low number of myeloma cells in the body.

    Stage II multiple myeloma

    In stage II multiple myeloma, there is a moderate number of myeloma cells in the body.

    Stage III multiple myeloma

    In stage III multiple myeloma, there is a large number of myeloma cells in the body.

    Other plasma cell neoplasms are staged differently than multiple myeloma.

    Isolated plasmacytoma of bone

    In isolated plasmacytoma of bone, one plasma cell tumour 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 tumour 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), the amount of M protein in the blood stays the same, less than 10% of the bone marrow is made up of plasma cells, and the patient has no signs of cancer.

    Refractory Multiple Myeloma and Other Plasma Cell Neoplasms

    Multiple myeloma and other plasma cell neoplasms are refractory when the number of plasma cells continues to increase even though treatment is given.

    Treatment Option Overview

    Key Points for This Section

    • There are different types of treatment for patients with multiple myeloma and other plasma cell neoplasms.
    • Eight types of standard treatment are used:
      • o Chemotherapy
      o Other drug therapy
      o High-dose chemotherapy with bone marrow or stem cell transplant
      o Biologic therapy
      o Radiation therapy
      o Surgery
      o Watchful waiting
      o Plasmapheresis
    • Other types of treatment are being tested in clinical trials. These include the following:
      • o New combinations of therapies

      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. Before starting treatment, patients may want to think about taking part in a clinical trial. 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.

      Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.

      Eight 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 the cells 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 antitumour effects in lymphomas and lymphoid leukaemias.

      Thalidomide

      Thalidomide is a drug that prevents the growth of new blood vessels into a solid tumour.

      Bisphosphonate therapy

      Bisphosphonates are substances that bind to the surface of damaged bones and reduce new bone damage, allowing the bone to rebuild. This will also reduce the amount of calcium released into the blood from bone damage.

      High-dose chemotherapy with bone marrow or stem cell transplant

      This treatment is a method of giving high doses of chemotherapy and radiation therapy 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 and radiation therapy are 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. 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 tumour 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.

      Other types of treatment are being tested in clinical trials. These include the following:

      New combinations of therapies

      Clinical trials are studying different combinations of biologic therapy, chemotherapy, steroid therapy, and thalidomide.

      Treatment Options for Multiple Myeloma and Other Plasma Cell Neoplasms

      Multiple Myeloma

      Patients without symptoms may not need treatment. When symptoms appear, treatment for any stage of multiple myeloma may include the following:

      • High-dose corticosteroid therapy.
      • Thalidomide therapy.
      • Combination chemotherapy.
      • High-dose chemotherapy, radiation therapy, and bone marrow or stem cell transplant.
      • Biologic therapy with monoclonal antibodies.
      • Radiation therapy for tumours of the spine.
      • A clinical trial combining thalidomide, a corticosteroid, and chemotherapy.
      • A clinical trial combining biologic therapy, corticosteroids, and/or a drug such as thalidomide.

      Treatment with bisphosphonate drug therapy may be given to slow bone loss and reduce bone pain. Treatment of amyloidosis is usually chemotherapy.

      Isolated Plasmacytoma of Bone

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

      Standard treatment of extramedullary plasmacytoma may include the following:

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

      Waldenström's Macroglobulinemia (Lymphoplasmacytic Leukaemia)

      Treatment of Waldenström's 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.

      Monoclonal Gammopathy of Undetermined Significance

      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.
      Refractory Plasma Cell Neoplasms

      Treatment of refractory plasma cell neoplasms may include the following:

      • Watchful waiting for patients whose disease is stable.
      • A different treatment than previously given.

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