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Mycosis Fungoides and the Sezary Syndrome General Information What are plasma cell neoplasms? Plasma cell neoplasms are diseases in which certain cells in the blood (called plasma cells) become cancer. Plasma cells are made by white blood cells called lymphocytes. The plasma cells make antibodies, which fight infection and other harmful things in the body. When these cells become cancer, they may make too many antibodies and a substance called M-protein is found in the blood and urine. There are several types of plasma cell neoplasms. The most common type is called multiple myeloma. In multiple myeloma, cancerous plasma cells are found in the bone marrow. The bone marrow is the spongy tissue inside the large bones in the body. The bone marrow makes red blood cells (which carry oxygen and other materials to all tissues of the body), white blood cells (which fight infection), and platelets (which make the blood clot). The cancer cells can crowd out normal blood cells, causing anemia (too few red blood cells). The plasma cells also may cause the bone to break down. The plasma cells can collect in the bone to make small tumors called plasmacytomas. Plasma cell neoplasms also can appear only as growths of plasma cells (plasmacytomas) in the bone and soft tissues, without cancer cells in the bone marrow or blood. Macroglobulinemia is a type of plasma cell neoplasm in which lymphocytes that make an M-protein build up in the blood. Lymph nodes and the liver and spleen may be swollen. If there are symptoms, a doctor will order blood and urine tests. If the tests are not normal, the doctor may do a bone marrow biopsy. During this test, a needle is inserted into a bone and a small amount of bone marrow is taken out and looked at under the microscope. The doctor can then tell what kind of cancer the patient has and plan the best treatment. X-rays also may be done to see whether the bones are affected. The chance of recovery (prognosis) depends on the kind of plasma cell neoplasm, and the patient's age and general health. Stage Information Stages of plasma cell neoplasms: Once plasma cell neoplasm has been found, more tests will be done to see how far the cancer has spread. This is called staging. Plasma cell neoplasms are grouped together depending on the type of plasma cell cancer that is found. The following stages are used for multiple myeloma: Multiple myeloma Stage I multiple myeloma Relatively few cancer cells have spread throughout the body. The number of red blood cells and the amount of calcium in the blood are normal. No tumors (plasmacytomas) are found in the bone. The amount of M-protein in the blood or urine is very low. There may be no symptoms of disease. Stage II multiple myeloma A moderate number of cancer cells have spread throughout the body. Stage III multiple myeloma A relatively large number of cancer cells have spread throughout the body. There may be one or more of the following: 1. A decrease in the number of red blood cells, causing anemia. 2. The amount of calcium in the blood is very high, because the bones are being damaged. 3. More than three bone tumors (plasmacytomas) are found. 4. High levels of M-protein are found in the blood or urine. The following groups are used to determine the treatment of plasma cell neoplasms that don't involve the bone marrow. Isolated plasmacytoma of bone Only a single plasma cell tumor is found in the bone without any other evidence of cancer. Patients may develop multiple myeloma at a later time. Extramedullary plasmacytoma Plasma cell tumors are found only outside the bone and the bone marrow in the soft tissues, usually the tonsils or tissues around the nose. Patients may develop multiple myeloma at a later time. Macroglobulinemia Plasma cells that produce a certain type of M-protein are found in the blood. Patients usually have swollen lymph nodes and spleen or liver. Monoclonal gammopathy of undetermined significance M-protein is found in the blood without symptoms or other signs of cancer. People with this condition may develop plasma cell neoplasms or cancer of the lymph system (lymphoma) at a later time. Refractory plasma cell neoplasms The plasma cells do not decrease even though treatment is given. Treatment Option Overview How plasma cell neoplasms are treated: There are treatments for all patients with plasma cell neoplasms. Three kinds of treatment are used: -chemotherapy (using drugs to kill cancer cells) -radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells -biological therapy (using the body's immune system to fight cancer) Surgery may be used in certain cases. Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body. Radiation therapy uses x-rays or other high-energy rays to kill cancer cells and shrink tumors. Radiation for plasma cell neoplasms usually comes from a machine outside the body (external radiation therapy). Biological therapy tries to get the body to fight cancer. It uses materials made by the body or made in a laboratory to boost, direct, or restore the body's natural defenses against disease. Biological therapy is sometimes called biological response modifier (BRM) therapy or immunotherapy. Bone marrow transplantation is used to replace the bone marrow with healthy bone marrow. First, all of the bone marrow in the body is destroyed with high doses of chemotherapy with or without radiation therapy. Healthy marrow is then taken from another person (a donor) whose tissue is the same as or almost the same as the patient's. The donor may be a twin (the best match), a brother or sister, or an unrelated person. The healthy marrow from the donor is given to the patient through a needle in the vein to replace the marrow that was destroyed. A bone marrow transplant using marrow from a relative or unrelated person is called an allogeneic bone marrow transplant. Another type of bone marrow transplant, called autologous bone marrow transplant, is being studied in clinical trials. To do this type of transplant, bone marrow is taken from the patient and treated with drugs to kill any cancer cells. The marrow is then frozen to save it. Next, the patient is given high-dose chemotherapy with or without radiation therapy to destroy all of the remaining marrow. The frozen marrow that was saved is then thawed and given back to the patient through a needle in a vein to replace the marrow that was destroyed. Another type of autologous transplant is called a peripheral blood stem cell transplant. The patient's blood is passed through a machine that removes the stem cells (immature cells from which all blood cells develop). The machine then returns the blood back to the patient. This procedure is called leukapheresis and usually takes 3 or 4 hours to complete. The stem cells are treated with drugs to kill any cancer cells and then frozen until they are transplanted back to the patient. This procedure may be done alone or with an autologous bone marrow transplant. A greater chance for recovery occurs if a doctor chooses a hospital which does more than five bone marrow transplantations per year. If the spleen is swollen, the doctor may take out the spleen in an operation called a splenectomy. If too many M-proteins build up in the blood, the patient's blood may need to be filtered through a special machine. This is called plasmapheresis. Treatment By Stage Treatment of plasma cell neoplasms depends on the type and stage of the disease, and the patient's age and overall health. Standard treatment may be considered because of its effectiveness in patients in past studies, or participation in a clinical trial may be considered. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to find better ways to treat cancer patients and are based on the most up-to-date information. Clinical trials are ongoing in most parts of the country for most stages of plasma cell neoplasms. Multiple Myeloma If there are no symptoms, treatment may not be needed. A doctor will follow the patient closely so treatment can be started if symptoms develop. If there are symptoms, treatment will probably be chemotherapy. Clinical trials are testing new chemotherapy drugs and dose regimens. Isolated Plasmacytoma of Bone Treatment will probably be external radiation therapy to the tumor. If other symptoms appear, patients may receive chemotherapy. Extramedullary Plasmacytoma Treatment may be one of the following: 1. External radiation therapy to the tumor. 2. Surgery to remove the tumor, usually followed by external radiation therapy. If other symptoms appear, patients may receive chemotherapy. Waldenstrom's Macroglobulinemia (Lymphoplasmacytic Leukemia) If there are no symptoms, treatment may not be needed. A doctor will follow the patient closely so treatment can be started if symptoms develop. If there are symptoms, treatment may be chemotherapy, biologic therapy, or immunotherapy. Clinical trials are testing new chemotherapy drugs and combinations of drugs with or without stem cell support or transplant. Monoclonal Gammopathy of Undetermined Significance A doctor will follow the patient closely to see if symptoms of plasma cell neoplasm or lymphoma develop. Refractory Plasma Cell Neoplasm Treatment will probably be chemotherapy. Clinical trials are testing new drugs and combinations of drugs. The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals. The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation. The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. 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