This information is provided by SSCHRC to help you understand Waldenström Macroglobulinemia and its management. It is written in a patient-friendly and medically accurate style.
Waldenström Macroglobulinemia (WM) is a rare, slow-growing type of non-Hodgkin lymphoma, which is a cancer affecting the immune system. Although the condition is currently considered incurable, patients generally respond well to treatment, allowing for successful long-term management.
WM originates from B-cell lymphocytes, a type of white blood cell in the immune system. The cancerous cells can accumulate and multiply in various organs, including the liver, spleen, and lymph nodes, causing them to swell. They also frequently build up in the bone marrow, which can lead to a crowding out of healthy, normal cells. This process can result in a drop in the levels of healthy blood cells, specifically:
A key feature of WM is that the cancerous cells often produce an excessive amount of a protein called Immunoglobulin M (IgM). When this protein reaches very high levels, it can cause the blood to become unusually thick, a condition known as hyperviscosity, which makes it difficult for blood to flow smoothly through the blood vessels.
While anyone can develop WM, the disease is most common in:
There is also an increased risk for people whose siblings, children, or parents have been diagnosed with WM.
Factors that increase the likelihood of developing Waldenström Macroglobulinemia include:
Important Note: It is important to remember that not everyone with these risk factors will develop the disease. If you have any concerns regarding your risk factors, you should discuss them with your doctor.
In some instances, people may have WM for several years without exhibiting any symptoms. When signs do appear, they are primarily caused by low blood cell counts or by the thickening of the blood. Symptoms can vary significantly among individuals.
As the disease progresses, symptoms may become more severe and include:
Note: Many people experiencing these symptoms do not have Waldenström Macroglobulinemia, as similar signs are shared with other conditions, including autoimmune diseases. Anyone with these symptoms should seek medical advice promptly.
Achieving an early and accurate diagnosis is critical for successfully managing WM. Given the rarity of the disease, it is essential to seek care from a team with specialised experience in both recognising and treating this condition. Experts at SSCHRC are among the most experienced in the country at identifying WM.
If your signs or symptoms suggest Waldenström Macroglobulinemia, your doctor will perform an examination and take a thorough medical history. One or more of the following tests may be used for diagnosis, and later, to monitor the effectiveness of treatment:
A biopsy is the only method to definitively diagnose Waldenström Macroglobulinemia. This involves removing a small tissue sample to be examined under a microscope for cancer cells.
Doctors use a needle to take a sample of bone marrow, typically from a large bone like the pelvis. Patients receive local anaesthesia, and some may also be given light sedation.
A small piece of tissue is removed from a lymph node, or sometimes the entire node, for microscopic examination.
These are used to detect and track the levels of abnormal proteins produced by WM cells. These are known as monoclonal proteins (M proteins or paraproteins) in the blood, and Bence Jones proteins in the urine. Blood tests are also performed to measure blood thickness, assess organ function, and conduct a complete blood count.
Used to look for cancer inside the body, locate tumours, and monitor the body's response to treatment. The most common imaging exams for WM include:
There is no routine screening protocol for Waldenström Macroglobulinemia. However, patients who have been diagnosed with MGUS or have a family history of WM should inform their primary care team so that they can be monitored closely for any developing symptoms.
Waldenström Macroglobulinemia is a slow-growing, chronic lymphoma. While a cure is not currently available, the disease can be managed successfully. A diagnosis of WM means you will require care for the rest of your life. Our expert team will collaborate with you to create a personalised treatment plan that controls the disease's progression while focusing on minimising side effects.
Due to its slow development, some patients may not require immediate treatment. In these cases, doctors will closely monitor the disease's status, and treatment will only begin if it progresses past a pre-determined threshold.
These drugs are used to kill cancer cells, control their growth, or alleviate disease-related symptoms. Chemotherapy may involve a single agent or a combination of two or more drugs. WM patients usually receive chemotherapy combined with monoclonal antibody drugs (a type of immunotherapy), a combination known as chemoimmunotherapy, which research has shown to be more effective than either treatment alone.
Administration: Given via IV infusions (usually around 30 minutes) and/or injections under the skin. Treatments typically last for a set duration, often about six months. Effective treatment can allow patients to remain in remission for several years before needing further intervention.
This is a form of immunotherapy where antibodies are designed to attach to specific proteins on the surface of cancer cells. This marks the cancer cells as a target, boosting the immune system's ability to fight the disease.
Administration: Typically given as an IV infusion, usually one infusion per treatment cycle. The first infusion is generally longer (several hours) and carries the highest chance of a negative reaction.
These drugs are specifically designed to stop or slow the growth and spread of cancer at a cellular level. They interfere with the specific molecules or cancer-causing genes that WM cells need to survive and multiply.
Administration: Given as pills, which patients take for as long as the treatment is effective. Targeted therapy can be used alone or combined with monoclonal antibodies.
This uses powerful, focused beams of energy to destroy cancer cells. It is sometimes employed to treat and eliminate a mass of cancer cells that is causing symptoms or potential damage to an organ.
Administration: Patients usually receive daily treatment until the mass is controlled, typically in 10 days or less. Each session lasts between 30 minutes to one hour. Lower radiation doses are often used for WM, making the treatment generally well-tolerated.
This procedure replaces cancerous bone marrow with new, healthy bone marrow stem cells. It is not a routine treatment for WM but is offered when other treatments have not successfully controlled the disease. Most WM stem cell transplants are autologous, meaning the healthy stem cells are collected from the patient themselves.
Administration: Usually follows an intense round of chemotherapy designed to kill existing bone marrow cells. WM patients typically stay in the hospital for approximately two weeks after the transplant.
If a patient develops symptoms due to excessively thick blood, this procedure can be performed to quickly relieve symptoms. Plasma is removed and replaced with normal plasma from a healthy donor. This provides immediate relief until chemotherapy or immunotherapy can destroy the WM cells causing the protein build-up.
Administration: The procedure can take several hours. IV lines are inserted into the patient's arms: one removes blood and sends it to a machine for plasma replacement, and the other returns the treated blood to the patient.
As WM is a slow-growing cancer that typically responds well to treatment, most patients are able to maintain their daily activities even while undergoing therapy.
Patients with WM have a heightened risk of developing another cancer; therefore, it is vital that they adhere to all standard cancer screening guidelines appropriate for their age and gender. Additionally, since the disease affects the immune system, patients should consult their doctor to ensure their vaccinations are up to date.