About 1,500 new cases of Waldenström macroglobulinemia are diagnosed each year in the United States, accounting for less than two percent of lymphomas. The disease usually affects older adults. This lymphoma is slow growing and is found mainly in the bone marrow, lymph nodes, and spleen.
It is different from other lymphomas because Waldenström lymphoma cells often make immunoglobulin M (IgM), a very large antibody. IgM circulates in the blood, sometimes causing it to become thick, similar to the consistency of syrup. When this happens, blood flow is decreased too many organs, which may cause problems with vision and neurological problems, such as a headache, dizziness, and confusion. In addition, patients with Waldenstrom’s may bleed easily.
Patients who do not have symptoms may initially be treated with watchful waiting. Thickening of the blood may be treated with a procedure called plasmapheresis, which removes the patient’s blood, passes it through a machine that eliminates the IgM, and then returns the blood to the patient. This may be combined with other treatments, including single-agent or combination chemotherapy. Biologic agents and stem cell transplant may also be used. Although it isn’t usually considered to be curable, patients may continue to be treated and achieve additional remissions.
Diagnosis of Waldenstrom Macroglobulinemia:
A diagnosis of WM may be suspected if blood test results show low blood counts or unusually high protein levels. To determine the presence and amount of IgM monoclonal proteins, an additional test called “serum protein electrophoresis” (SPEP) will be performed. SPEP is used to identify the presence of abnormal proteins, to identify the absence of normal proteins and to determine increases and decreases of different groups of proteins in serum. This test is typically ordered to detect and identify excessive production of specific proteins (immunoglobulins). All five types of immunoglobulin (IgG, IgA, IgM, IgE, or IgD) are measured by this test. An excessive production of a monoclonal immunoglobulin may be shown on lab results as a spike on a graph. Generally, IgM protein levels greater than 3 grams per deciliter (g/dL) are an indication of WM.
- Immunophenotyping. This is a method used to identify a specific type of cell in a sample of blood or marrow cells to determine if the abnormal lymphocytes are B cells or T cells. The lymphocytes associated with WM are B cells and are also characterized by the cell markers (antigens on the surface of the cell) CD19, CD20, CD22, CD79, and FMC7. Expressions of CD5, CD10, and CD23 may be found in 10 to 20 percent of WM cases.
- Flow cytometry. In this test, cell properties are measured using a light-sensitive dye and a laser beam or other type of light. The test is often used to look at markers on the surface of cells or inside the lymphocytes. Flow cytometry has become increasingly important in helping doctors to determine a patient’s exact type of lymphoma.
- An analysis of urine collected over 24 hours to detect elevated levels of protein in the urine.
Treatment for Waldenstrom Macroglobulinemia:
There are several treatment options available to prevent or control symptoms of WM and improve the quality of life of patients. Not all newly diagnosed WM patients will need immediate treatment. Twenty-five percent of WM patients are asymptomatic (have no symptoms) at diagnosis, and 50 percent of those patients will not require therapy within three years. Asymptomatic patients are medically observed in an approach called “watchful waiting” or “watch-and-wait.” Active treatment for these patients only begins if symptoms develop. In the past, increases in IgM levels were used as the benchmark to begin treatment. However, it was determined that the IgM level alone does not accurately reflect the tumor burden or prognosis in WM. To date, there is no evidence suggesting that treatment of asymptomatic WM patients provides a greater survival benefit than treatment of patients who begin therapy once symptoms appear
Alkylating agents—This class of chemotherapy drugs includes chlorambucil (Leukeran®), cyclophosphamide (Cytoxan®), melphalan (Alkeran®) and bendamustine (Treanda®). Treanda is FDA approved to treat chronic lymphocytic leukemia (CLL) and indolent non-Hodgkin lymphoma (NHL) that has progressed during or within 6 months of treatment with rituximab (Rituxan®) or a rituximab-containing regimen. These drugs directly damage the DNA of cells. People who are candidates for stem cell transplantation should not be treated with alkylating agents—with the exception of cyclophosphamide—because these drugs are likely to decrease the production of functioning red cells, white cells and platelets.
Based on the favorable outcomes reported in recent studies, the use of combination therapy (treatment with two or more drugs) is increasingly being favored for previously untreated patients or for those with relapsed WM. Some examples of combination therapies currently used in the treatment of WM patients include l BDR: Bortezomib (Velcade), dexamethasone and rituximab (Rituxan) l RCD: Cyclophosphamide (Cytoxan), dexamethasone and rituximab (Rituxan) l R-CHOP: Cyclophosphamide (Cytoxan), doxorubicin (Adriamycin®), vincristine (Oncovin®), prednisone and rituximab (Rituxan) l CPR: Cyclophosphamide (Cytoxan), prednisone, rituximab (Rituxan) l VR: Bortezomib (Velcade) and rituximab (Rituxan) l FR: Fludarabine (Fludara) and rituximab (Rituxan) l TR: Thalidomide (Thalomid) and rituximab (Rituxan). Clinical trials are underway to determine the long-term results and adverse side effects of combination therapy strategies in the treatment of WM. Certain long-term or late effects have been associated with the use of alkylating agents and purine nucleoside analogs, such as transformation to a more aggressive WM and development of a myelodysplastic syndrome or acute myeloid leukemia. Patients should speak to their doctors about the benefits and risks of any treatment. See the free LLS fact sheet Long-Term and Late Effects of Treatment in Adults for additional information about potential long-term effects of these and other drug treatments
Radiation therapy is used infrequently to treat WM patients because of the toxic effects of this treatment on older patients. It may be used in the rare occurrence of bony lesions.
Stem cell transplantation is being studied in clinical trials for the treatment of WM. This therapy is rarely used for newly diagnosed patients unless they have multiple high-risk features, but high-dose chemotherapy with stem cell transplantation is an option for some relapsed and/or refractory patients, especially younger patients who have had multiple relapses or who have the primary refractory disease. There are two main types of stem cell transplantation: autologous and allogeneic. Autologous stem cell transplantation is the type most often used in WM patients. This procedure uses the patient’s own stem cells to restore blood cell production after intensive chemotherapy. Based on data from a number of clinical trials, autologous stem cell transplants are showing high response rates even in patients whose disease was refractory to several regimens of standard chemotherapy. Allogeneic stem cell transplantation (infusion of donor stem cells into a patient) has more risks and side effects than autologous stem cell transplantation and is usually reserved for younger patients with advanced disease who have failed to respond, or no longer respond, to other treatment options. A newer approach to allogeneic stem cell transplantation, called “reduced-intensity transplantation” or “nonmyeloablative transplantation,” uses lower doses of chemotherapy or radiation therapy. This type of transplant may be an option for older and sicker patients who are not responding to other treatments. Talk to your doctor about whether stem cell transplantation is a treatment option for you.
2nd International Conference on Hematology and Oncology
London UK | August23-25, 2018
Conference URL: https://goo.gl/fMKgfH