Waldenstrom’s disease is a rare, slow-spreading type of form of non-Hodgkin’s lymphoma.
These are cancers that start in white blood cells, called lymphocytes. Lymphocytes are part of your immune system, which defends your body from pathogens and fights off infections.
Waldenstrom’s disease is also known as:
- Waldenstrom’s macroglobulinemia
- lymphoplasmacytic lymphoma
- primary macroglobulinemia
To understand Waldenstrom’s disease, it’s important to understand how the body’s immune system works.
The immune system produces cells that protect your body against infection. One such cell is the B lymphocyte, also known as a B cell.
B cells are a form of white blood cell that responds to infection by changing into another type of cell called a plasma cell. Plasma cells make proteins called antibodies, or immunoglobulins, that help the body attack invading diseases.
Waldenstrom’s disease is characterized by an excess number of abnormal white blood cells in the bone marrow. These cells are called lymphoplasmacytic, or WM, cells, and they have characteristics of both lymphocytes and plasma cells.
WM cells produce an excess amount of a type of antibody called immunoglobulin M, or IgM. When this happens, your blood will become thicker. This is known as hyperviscosity, a condition that makes it difficult for all of your organs and tissues to function properly.
Additionally, the growth of WM cells can lead to healthy blood cells being “crowded out,” which leads to low levels of red blood cells — a condition called anemia.
WM cell growth can also cause a reduction in white blood cells, which can make you more susceptible to infection, as well as a reduction in blood platelets, which can lead to increased bleeding and bruising.
The symptoms of Waldenstrom’s disease will vary based on the severity of your condition. In some instances, people with this condition have no symptoms, and the cancer is only found after a person has routine blood tests done.
Symptoms of Waldenstrom’s disease can be similar to other types of non-Hodgkin’s lymphoma.
Some common symptoms of this disease are:
- Weakness and fatigue. This is one of the most common symptoms and is typically caused by anemia or the thickening of the blood due to lgM buildup.
- Loss of appetite and weight loss. Appetite loss is a side effect of WM and can lead to unexplained weight loss.
- Fever and sweats. WM can cause fevers and drenching night sweats, even if a person doesn’t have an infection.
- Neuropathy. Numbness or loss of feeling in the feet and legs can happen if abnormal antibodies cause nerve damage outside the brain.
Some less common symptoms include:
- Abnormal bleeding. Excess levels of abnormal igM can damage blood vessels, leading to abnormal bleeding like nosebleeds and bleeding gums.
- Blood circulation problems. When WM causes hyperviscosity syndrome, thickened blood can cause poor circulation in the brain and body, causing headache, confusion, dizziness, and stroke-like symptoms.
- Swollen lymph nodes. These appear as lumps under the skin around the neck, groin, or armpits.
- Swollen abdomen. When WM causes the spleen or liver to become enlarged, the belly may appear swollen.
- Heart problems. High levels of lgM can damage tissue and muscles in the heart and cause the heart to have to work harder to pump blood, which can lead to congestive heart failure.
- Kidney problems. High levels of lgM can cauase damage to the kidneys.
- Digestive symptoms. lgM buildup in the intestines can lead to GI symptoms like diarrhea, bloody stools, and poor absorption of vitamins.
- Sensitivity to cold. In some people, lgM and other proteins react to cold temperatures to form clumps that block blood flow in areas of the body exposed to cold, leading to pain in the hands and feet or loss of color in fingers and toes exposed to the cold.
- Vision problems. Excess bleeding or poor circulation around blood vessels inside the eyes may cause blurred vision or blind spots.
- Infections. High levels of lgM can reduce the body’s ability to produce healthy antibodies, making the body more susceptible to infection.
Waldenstrom’s disease develops when your body overproduces IgM antibodies. The cause of this disease is unknown, though it is believed to be due to gene mutations that develop throughout a person’s life.
Risk factors for Waldenstrom’s disease
There are certain factors that may increase a person’s risk of developing Waldenstrom’s disease. However, Waldenstrom’s disease is relatively rare, and most people who have these risk factors will not develop it.
Risk factors include:
- Sex. People assigned male at birth are more likely to develop Waldenstrom’s disease than people assigned female.
- Age. A person’s risk of developing Waldenstrom’s disease increases with age.
Research demonstratesthat Waldenstrom’s disease has a higher incidence rate among white people than among Black people or other racial groups.
- Heredity. Researchers have determined that certain genes may play a role in some people who develop Waldenstrom’s disease.
- Hepatitis C. People with chronic hepatitis C may be more likely to develop Waldenstrom’s disease than people without. But not all studies have found a connection between the two.
- Some autoimmune disorders. People with certain autoimmune disorders, including Sjögren’s syndrome, may be at an increased risk of developing Waldenstrom’s disease.
To diagnose this disease, your doctor will start by performing a physical exam and ask you about your health history. Your doctor may check for swelling in your spleen, liver, or lymph nodes during the exam.
If you have symptoms of Waldenstrom’s disease, your doctor may order additional tests to confirm your diagnosis. These tests may include:
- blood tests to determine your level of IgM and to evaluate the thickness of your blood
- a bone marrow biopsy
- CT scans of bones or soft tissue
- X-rays of bones or soft tissue
CT scan and X-ray of the bones and soft tissues are used to differentiate between Waldenstrom’s disease and another type of cancer called multiple myeloma.
There’s no cure for Waldenstrom’s disease. However, treatment can be effective at managing your symptoms.
Treatment for Waldenstrom’s disease will depend on the severity of your symptoms. Not everyone needs to be treated right away, particularly people who don’t have serious or bothersome symptoms.
People who don’t have symptoms but have been diagnosed with Waldenstrom’s should be monitored closely and treated if necessary.
If you have symptoms of the disease, there are several different treatments your doctor may recommend. These include:
Chemotherapy is a treatment that destroys cells in the body that grow quickly. Chemotherapy for Waldenstrom’s disease is designed to attack the abnormal cells producing the excess IgM.
A doctor may administer chemotherapy treatment as a pill or intravenously, which means through your veins. Chemotherapy is given in cycles, with rest periods in between to allow the body time to recover.
Many types of chemo drugs are used to treat Waldenstrom’s, including:
- Alkylating agents: cyclophosphamide (Cytoxan), bendamustine (Treanda)
- Purine analogs: fludarabine (Fludara,) cladribine (2-CdA, Leustatin)
- Other chemo drugs: vincristine (Oncovin), doxorubicin (Adriamycin)
Chemotherapy drugs may sometimes be used in combination with other drugs and therapies.
Immunomodulators are a type of treatment that attacks cancer cells by starving them from the body’s blood supply, enhancing immune cells, blocking cancer cell and bone marrow interactions, and directly killing cancer cells.
This type of treatment is given as an oral medication in three different options:
- thalidomide (Thalomid)
- lenalidomide (Revlimid)
- pomalidomide (Actimid or Pomalyst)
Plasmapheresis, or plasma exchange, is a procedure in which excess IgM immunoglobulins in plasma are removed from a person’s blood by a machine. The remaining plasma is then combined with plasma from a donor and returned to the body.
This type of therapy is used in cases of hyperviscosity syndrome, where the blood becomes very thick.
While plasmapheresis can effectively and quickly reduce lgM levels, it doesn’t treat the cause of high lgM, so levels will rise again without treatment like chemotherapy or radiation therapy.
It’s often given to help lower lgM levels until other treatments begin to work.
Biological therapy (Immunotherapy)
Immunotherapy, also called biological therapy, is used to boost the immune system’s ability to fight cancer. It can be used with chemotherapy. There are different types of immunotherapy, including:
- Monoclonal antibodies. These are human-made versions of antibodies, which are proteins made by the immune system to fight infection.
- Immunomodulating drugs. These are thought to fight cancer by boosting the immune system.
- Cytokines. These are proteins normally made by white blood cells to power your immune system.
Corticosteroids are often given in combination with other therapies or before administering monoclonal antibodies to help reduce and prevent allergic reactions.
Prednisone and dexamethasone are the most common corticosteroids given to patients with Waldenstrom’s disease.
Targeted drug therapy
Targeted drug therapies were developed to prevent cancer cells from changing and spreading. They target specific proteins and enzymes that cancer cells use to grow and divide, and sometimes work when chemotherapy drugs don’t.
Examples of targeted drug therapies include:
- Bruton’s tyrosine kinase (BTK) inhibitors, commonly ibrutinib (Imbruvica) and zanubrutinib (Brukinsa)
- proteasome inhibitors like bortezomib (Velcade), carfilzomib (Kyprolis), and ixazomib (Ninlaro)
- mTOR inhibitors
Stem cell transplant
Stem cell transplants aren’t a common treatment for Waldenstrom’s disease but may be an option for younger people when cancer is resistant to other treatments.
A person’s body can only take a certain amount of chemotherapy or radiation therapy before their bone marrow, where new blood cells are made, becomes severely damaged. This can lead to a low blood cell count and cause dangerous infections and bleeding.
Stem cell transplants, which use stem cells from a person’s blood, can help to create new blood cells in a person’s bone marrow, enabling them to receive more chemo- or radiation therapy.
Radiation therapy, which uses beams of high energy radiation to kill cancer cells, is not often used to treat Waldenstrom’s disease. In rare cases, it may be used to shrink a spleen or lymph node that has become enlarged.
Following your diagnosis, you should also ask your doctor about
Clinical trials are often used to test new treatments or to investigate new ways to use existing treatments.
The National Cancer Institute may be sponsoring clinical trials that may provide you with additional therapies to treat the disease.
Is Waldenstrom’s disease curable?
There’s currently no cure for Waldenstrom’s disease, but there are treatments available to help manage it, often for long periods of time.
Is Waldenstrom’s disease chronic?
Yes, Waldenstrom’s disease is chronic. It’s a rare form of non-Hodgkin’s lymphoma.
What are the late stages of Waldenstrom’s disease?
There’s no standardized staging system for Waldenstrom’s disease. When determining a person’s prognosis and disease severity, doctors look at multiple factors, including:
- blood cell counts
- the amount of lgM in the blood
- the amount of beta-2 microglobulin protein in the blood
People with Waldenstrom’s who are older, are anemic, or have low blood platelets generally have a poorer prognosis.
How long can someone live with Waldenstrom’s?
According to data from the
The 5-year survival rates for different risk groups are:
- 87 percent for the low risk group
- 68 percent for the intermediate risk group
- 36 percent for the high risk group
You should know that survival rates are based on previous outcomes of large numbers of people. They’re estimates and can’t predict what will happen in any particular case.
Additionally, the 5-year survival rate for Waldenstrom’s disease dramatically increased between the 1980s and 2010s. As treatments improve over time, people may have a better outlook.
If you’re diagnosed with Waldenstrom’s disease, the outlook will depend on the progression of your condition.
The disease progresses at different rates depending on the person. Those who have a slower disease progression have a longer survival time compared with those whose disease progresses more quickly.
With the help of your doctor and medical team, you can treat and manage your symptoms as well as explore new clinical trials.
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