A slow-growing type of cancer that affects blood cells and occurs quite rarely is called Waldenstrom Macroglobulinemia (WM). Sometimes, this type of cancer is called lymphoplasmacytic lymphoma. It often happens when a person develops genetic changes (mutations) in the B cells. Although it is not possible to cure this condition, treatments focus on lessening the symptoms and preventing serious complications.
This rare cancer is a type of non-Hodgkin lymphoma, and it only affects approximately 3 to 4 people out of 1 million in the U.S. It occurs when B cells (also known as immune cells) in the bone marrow develop DNA changes. Furthermore, cancerous cells may release an abnormal protein known as immunoglobulin M (IgM). When this protein becomes too much in the bloodstream, it may thicken the blood, and it turns into a syrup-like liquid. It is called hyperviscosity syndrome, and it causes the blood to flow more slowly than usual, which leads to severe symptoms.
In general, it is not possible to cure this type of cancer. Usually, WM develops gradually, and people live many years after diagnosis, especially with proper treatment after diagnosis.
Symptoms
Approximately 25% of people who are diagnosed with this type of cancer do not experience symptoms. The symptoms usually appear slowly because the cancer develops gradually over time. Check below some symptoms of WM:
- Weakness
- Fatigue (extreme tiredness)
- Fever
- Night sweats
- Appetite changes
- Unusual weight loss
- Confusion
- Enlarged lymph nodes, spleen, or liver
- Peripheral neuropathy symptoms (such as tingling or numbness in the fingers or toes)
- Thickened blood symptoms (including nosebleeds, bleeding gums, dizziness, headaches, and vision changes)
If any of the symptoms listed above occur, immediately visit a doctor.
Causes
This type of cancer is often found during examinations and tests done for other reasons. Moreover, roughly 90% of people who are diagnosed with WM have a genetic mutation involving the MYD88 gene. About 40% of people have a mutation in their CXCR4 gene. Both genes contribute to the abnormal growth and multiplication of the abnormal cells.
However, previous gene mutations are not inherited. It means the changed genes do not pass from biological parents to biological children during pregnancy. MYD88 and CXCR4 gene mutations occur during a lifetime. Nowadays, researchers are trying to find what exactly causes these gene mutations.
Risk Factors
While healthcare providers cannot identify the exact cause of WM, they have identified some factors that could elevate your risk of developing it. Check below some examples:
- Age – WM is mostly diagnosed in people over 65 years old.
- Race – White people are more likely to develop this type of cancer.
- Sex – Men are at increased risk of developing WM than women.
- Medical history – Those who have hepatitis C, AIDS, Sjögren’s syndrome, or MGUS are at higher risk of developing WM. While MGUS is a condition that contributes to WM, not everyone with this condition develops it.
- Family history – If you have a parent or sibling with WM or other types of lymphoma, your risk of developing it significantly increases.
What Are The Possible Complications of Waldenstrom Macroglobulinemia?
People with WM may also experience some complications, especially if they do not have symptoms and do not get treatment. Examples include:
- Amyloidosis – This is a condition that causes faulty proteins to build up in certain organs such as the heart, lungs, or kidneys.
- Cryoglobulinemia – When it occurs, some blood proteins react to cold conditions and begin to build up in the hands and feet. It may also lead to cyanosis (pain and blue hands and feet).
- Hyperviscosity syndrome
- Bing-Neel syndrome
- Neuropathy
- Vasculitis
- Organ infiltration
- Autoimmune hemolysis
- Anemia
- Kidney disease
This article does not contain all possible complications of WM. However, you should see a doctor if you suspect you are developing any of the previous complications. Without treatment, these complications can be fatal.
How to Prevent Waldenstrom Macroglobulinemia?
However, it is not possible to prevent this condition. However, you can consider some steps that may reduce the risk of developing it. For example, manage health conditions that increase the risk of WM, including hepatitis C and others. You can also adopt lifestyle changes that may help reduce the symptoms of these conditions.
To prevent hepatitis C, you should avoid injection drug use, practice safe sex, and get immediate treatment to prevent serious complications.
Diagnosis
Usually, physicians perform multiple tests to confirm the condition. The following tests may also help exclude certain health conditions that cause similar symptoms to WM. Check below some tests:
- Urine and blood tests – These tests are done to check for low blood cell count and abnormal IgM proteins that indicate WM.
- Imaging tests – Healthcare professionals usually perform CT (computed tomography) and PET (positron emission tomography) scans to check for irregularities linked to WM. These include enlarged organs or lymph nodes and others.
- An eye examination – During this procedure, doctors check for bleeding in the back of the eye because it is a sign of a thickened blood vessel.
- Bone marrow biopsy – This test involves the removal of a small sample of bone marrow for testing. It helps identify cancerous cells.
Treatment
Doctors commonly recommend different treatments for people with WM. It depends on the severity, location, and type of cancer, your age, existing health problems (such as hepatitis C), and your preferences. Check below some treatments often recommended by doctors:
- Watchful waiting – Not everyone who develops WM needs treatment. Sometimes, people may not experience symptoms for years.
- Plasmapheresis (plasma exchange) – This treatment involves a specific machine that helps filter abnormal IgM protein from the plasma. It helps reduce the symptoms caused by thickened blood.
- Immunotherapy – This treatment involves medications that boost the immune system to find and destroy cancerous cells. However, cancer cells may produce some substances that help them hide from the immune system. Immunotherapy helps boost the immune system. Usually, doctors recommend Rituximab.
- Chemotherapy – This is a cancer treatment that involves strong medicines that kill cancer cells throughout the body. It is often recommended in advanced stages of cancer or when other treatments do not work. Doctors may prescribe chemotherapy along with immunotherapy to improve the effectiveness of the treatment.
- Corticosteroids – Physicians may recommend Dexamethasone to reduce the adverse reactions caused by cancer treatments.
- Targeted therapy – This treatment involves medications that target and block specific proteins in cancer cells, causing them to die. Physicians often prescribe Ibrutinib and Zanubrutinib. Both medications are approved by the FDA (Food and Drug Administration) for WM treatment.
- Stem cell transplant – Sometimes, people need this treatment to replace damaged bone marrow with healthy bone marrow. However, this treatment is not an option for everyone who develops WM.
Frequently Asked Questions
What is the life expectancy of Waldenstrom?
Some studies showed that there are a lot of people who live between 14 to 16 years after diagnosis. However, most diagnosed cases are in older people, and their survival time is usually very close to the average for people of their age.
What is the best treatment for Waldenstrom macroglobulinemia?
One of the most effective treatments for people with WM is chemotherapy. It uses a combination of strong drugs, often given intravenously (IV), to destroy cancer cells throughout the body. Sometimes, doctors may recommend immunotherapy along with this treatment.
What causes death with Waldenstrom?
The primary causes of death in people with WM include progression of the disease, transformation to an aggressive form of lymphoma, or serious complications caused by cancer treatments. If you have additional questions, ask your healthcare provider.
