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What is Waldenström’s macroglobulinemia?
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Waldenström’s macroglobulinemia, or lymphoplasmacytic lymphoma, is an uncommon type of non-Hodgkin’s lymphoma that is formed when cancer cells create significant amounts of macroglobulin.
The condition originates in B lymphocyte cells, which change into plasma cells when responding to infection within the body. Immunoglobulins then are produced by plasma cells to help combat disease-causing germs.
Patients with Waldenström’s macroglobulinemia have cancer cells that resemble that of multiple myeloma (plasma cells) and non-Hodgkin’s lymphoma (lymphocytes). The combination of features of both plasma cells and lymphocytes are known as lymphoplasmacytoid.
Risk factors
Although several factors increase a person’s risk for developing Waldenström’s macroglobulinemia, most people with these risk factors never go on to develop the condition.
For example, monoclonal gammopathy of undetermined significance — an abnormality of antibody-making cells — is associated with Waldenström’s macroglobulinemia.
Similar to Waldenström’s macroglobulinemia, monoclonal gammopathy of undetermined significance occurs when abnormal cells in the bone marrow create significant amounts of monoclonal protein. The condition does not cause health problems. However, 1% to 2% of people go on to develop Waldenstrom macroglobulinemia, multiple myeloma, lymphoma or amyloidosis.
Other risk factors for Waldenström’s macroglobulinemia include older age, male sex and white race. Heredity also is a factor. Research has shown people with hepatitis C or other autoimmune diseases, including Sjögren syndrome may be at increased risk for Waldenström’s macroglobulinemia.
Individuals with Waldenström’s macroglobulinemia are at greater risk for secondary malignancies than those in the general population, study results show.
Symptoms
There are many symptoms of Waldenström’s macroglobulinemia. They include excess bleeding, vision impairment and issues with the nervous system stem from the buildup of monoclonal proteins.
Patients may also experience anemia, low white blood cell counts and low numbers of platelets due to the cancer cells that grow in bone marrow and crowd the growth of normal cells that create blood cells. Patients may feel weak, their bodies may find it difficult to fight infection, and they may experience increased bleeding and bruising.
There also is a chance for lymphoma cells to grow in the organs, causing them to swell and potentially cause abdominal pain.
There are no widely recommended screening tests to identify people with Waldenström’s macroglobulinemia who do not experience any symptoms. However, cases are identified early when people go to the doctor due to symptoms they are experiencing or when patients undergo blood tests for other reasons.
Treatment and outcomes
Not all patients with Waldenström’s macroglobulinemia require treatment at first. Those who do not experience symptoms can be monitored closely and undergo treatment if necessary.
Treatments for Waldenström’s macroglobulinemia include chemotherapy, immunotherapy, high-dose chemotherapy and stem cell transplant, plasmapheresis, radiation therapy and other targeted therapies.
Chemotherapy and immunotherapies are the two primary treatment options. Recent research suggests various newer therapies are effective against Waldenström’s macroglobulinemia, but no single standard treatment exists for all patients.
Because current treatment options for patients with Waldenström’s macroglobulinemia often do not result in cure, patients often are treated for a period of time. Treatment is stopped, and then it is resumed when the condition returns.
However, a population-based study published earlier this year showed outcomes for patients with Waldenström’s macroglobulinemia have improved in the past 10 years.
Looking ahead
Researchers are trying to better understand the alterations in DNA and how they cause normal lymphocytes to develop into Waldenström’s macroglobulinemia cells. Cancer cells in Waldenström’s macroglobulinemia appear to have changes in the MYD88 and CXCR4 genes, which have been linked to a greater chance for the condition to cause symptoms, require treatment and affect survival. New therapies targeting cells with these gene changes are undergoing testing in early clinical trials.
Other clinical trials are examining newer therapies that have already shown promise against Waldenström’s macroglobulinemia. These include mTOR inhibitors, proteasome inhibitors, histone deacetylase inhibitors, Bruton’s tyrosine kinase inhibitors, PI3K inhibitors and aurora kinase inhibitors.
Additional research is focused on blocking the function of IL-6 that helps cause the bone destruction observed in myeloma. This may lead to new treatment options for patients with Waldenström’s macroglobulinemia.
Other areas of research include the continual improvement in bone marrow and peripheral blood stem cell transplant methods, as well as ways to boost immune reaction against Waldenström’s macroglobulinemia with vaccines.
More information is available at these websites:
www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/waldenstrom-macroglobulinemia-w-m
www.mayoclinic.org/diseases-conditions/waldenstrom-macroglobulinemia/basics/definition/CON-20036938
www.lymphoma.org/site/pp.asp?c=bkLTKaOQLmK8E&b=6300163