Waldenstrom Macroglobulinemia

Overview

What is Waldenstrom macroglobulinemia (WM)?

WM is a rare type of blood cancer that involves having abnormal lymphocytes (the main cells of lymphoid tissue) in the bone marrow or in lymph nodes. These cells produce abnormally high amounts of a certain type of antibody (immunoglobulin M, or IgM), which is known as a macroglobulin. Each antibody made by the abnormal cells is the same, so it is called a monoclonal protein, or just an M protein. The buildup of this M protein in the body can lead to many of the symptoms of WM, including excess bleeding, problems with vision, and nervous system problems.

WM is a slow-growing cancer that doctors classify as a non-Hodgkin lymphoma. It is sometimes called lymphoplasmacytic lymphoma. It usually occurs in people over the age of 60 and can be life-threatening. It is more common in men than women.

How common is Waldenstrom macroglobulinemia (WM)?

Doctors diagnose some 1,000-1,500 new cases per year. WM occurs more often in men than women, and more often in white people than other ethnic groups.

What are the risk factors for Waldenstrom macroglobulinemia (WM)?

People at higher risk for WM include those who:

Symptoms and Causes

What causes Waldenstrom macroglobulinemia (WM)?

Doctors do not know what causes WM but believe it involves a gene mutation. Recent research has found that WM cells have a mutation (change) in a gene known as MYD88, which normally helps immune system cells signal each other and helps keep them alive.

Having a family member with WM or other lymphomas may increase your risk of developing it, but often it’s not clear exactly how having these increases the risk The US Department of Veterans Affairs considers non-Hodgkin lymphoma in general to be linked to exposure to Agent Orange.

What are the symptoms of Waldenstrom macroglobulinemia (WM)?

Some cases of WM involve no symptoms. Doctors discover some cases when they are examining people for other reasons.

When WM does cause symptoms, the most common signs include:

  • Anemia, or a condition of not having enough red blood cells or hemoglobin. Hemoglobin moves oxygen around the body.
  • Fever
  • Loss of appetite
  • Night sweats
  • Weakness
  • Weight loss
  • Fatigue
  • Neuropathy

Diagnosis and Tests

How is Waldenstrom macroglobulinemia (WM) diagnosed?

Doctors use several tests to diagnose WM. They include:

  • Blood and urine tests: A doctor examines samples of these substances to identify signs of WM including low blood cell counts and high levels of immunoglobulin M (IgM).
  • Bone marrow biopsy: Using a needle inserted into the bone, a doctor removes a sample of marrow (a soft substance inside the bone that produces white blood cells). A laboratory examines the sample under a microscope to look for lymphoma cells.
  • Imaging tests: These tests that take pictures of the inside of your body, including X-rays and CT scans, can show signs of WM such as enlarged lymph nodes.

Management and Treatment

How is Waldenstrom macroglobulinemia (WM) managed or treated?

If you do not have any symptoms of WM, your doctor may not start treatment right away. This approach is called watchful waiting. People who don’t have symptoms may not need treatment for years.

When treatment is necessary, it varies according to the patient. Treatment options for WM include:

  • Biological therapy: Using the body’s own immune system to destroy or slow the growth of WM cells
  • Chemotherapy: Taking medications by mouth or by injection, so that they spread throughout the body to destroy cancer cells
  • Plasmapheresis (plasma exchange): Filtering immunoglobulin M (IgM) proteins out of the plasma (liquid part of the blood), using a special machine
  • Radiation therapy: Using strong X-rays or other radiation to destroy cancer cells
  • Stem cell transplant (also called bone marrow transplant): Infusing the body with stem cells (cells that form new blood cells) after using high doses of chemotherapy to destroy the bone marrow affected by WM
  • Targeted therapy: Using drugs that target specific changes happening inside cancer cells, without harming healthy cells

What complications are associated with Waldenstrom macroglobulinemia (WM)?

People with WM are at an increased risk of developing other types of cancer of the blood and other tissues. Other complications of WM include an enlarged (swollen) liver or spleen, and fatigue (extreme tiredness) due to anemia (lower than normal amounts of red blood cells). People with WM might also see a slow loss of kidney function.

When too much immunoglobulin M (IgM) collects in the blood, it can cause circulation problems. The related complications and their symptoms include:

  • Amyloidosis: Buildup of abnormal protein in organs including the heart, lungs and kidneys that can lead to problems in those organs
  • Cryoglobulin: Clumping of IgM in the hands and feet that can cause pain and blue or white discoloration (Raynaud phenomenon)
  • Hyperviscosity syndrome: This is a very serious presentation, and you should seek immediate medical evaluation. In WM, the thickening of the blood can cause circulation problems. In severe cases, this syndrome can lead to brain damage similar to a stroke. Symptoms include:
    • Bleeding in the mouth or nose
    • Blurred vision or double vision
    • Dizziness
    • Headache
    • Problems with coordination

Prevention

Can Waldenstrom macroglobulinemia (WM) be prevented?

No, you cannot prevent WM.

Outlook / Prognosis

What is the prognosis (outlook) for people with Waldenstrom macroglobulinemia (WM)?

WM is not curable but is very treatable. It can be life-threatening if left untreated. Most of the patients respond well to treatment. In some people, treatment eliminates the condition for a long time, even years. Other people may need ongoing treatments to manage it. Your doctor will tell you more about your specific case and prognosis. The gene mutation status at the time of diagnosis is also a factor in your outlook.

Living With

When should I call the doctor about Waldenstrom macroglobulinemia (WM)?

Contact your doctor if you experience signs or symptoms of WM. If you have WM, you should contact your doctor if:

  • You have signs of any type of infection
  • You are extremely tired
  • You have unusual bruising
  • You have confusion or other mental symptoms
  • You have any symptom that is new or is bothering you

What questions should I ask my doctor if I have Waldenstrom macroglobulinemia (WM)?

If you have WM, you may want to ask your doctor:

  • How serious is WM, and how will it affect my life?
  • What are my short-term and long-term prognosis?
  • Do I need treatment right away?
  • What type of treatment do you recommend?

When can I go back to my regular activities if I have Waldenstrom macroglobulinemia (WM)?

The time it takes to go back to your usual activities varies according to your individual treatment plan. In many cases, treatment for WM has a delayed response that may not appear until several months after treatment ends. Your doctor will talk to you about when you can return to your regular activities.

Resources

What resources exist for people with Waldenstrom macroglobulinemia (WM)?

Here are just a few resources to consider:

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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy

Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy