Overview

Waldenstrom macroglobulinemia (mak-roe-glob-u-lih-NEE-me-uh) is a rare cancer of white blood cells. It may be shortened to WM. It is a type of non-Hodgkin lymphoma called lymphoplasmacytic lymphoma (LPL). About 1,000 to 1,500 people are diagnosed with Waldenstrom macroglobulinemia each year in the United States. It accounts for about 1% of non-Hodgkin lymphomas.

In Waldenstrom macroglobulinemia, some white blood cells change and become cancer cells. These cells can build up in bone marrow, the soft tissue inside bones where blood cells are made. They can crowd out healthy blood cells. Cancer cells also may build up in lymph nodes, the spleen and other parts of the body.

Waldenstrom macroglobulinemia cells make a protein called immunoglobulin M (IgM). IgM can build up in the blood. Too much IgM can make blood too thick and slow blood flow. This can harm organs.

Waldenstrom macroglobulinemia can be serious, but it often worsens slowly. Some people do not need treatment right away. When treatment is needed, it can control symptoms and complications.

Waldenstrom macroglobulinemia and multiple myeloma

Waldenstrom macroglobulinemia and multiple myeloma are different blood cancers that occur in the bone marrow. Waldenstrom macroglobulinemia is a lymphoplasmacytic lymphoma affecting B cells and makes IgM. Multiple myeloma is a cancer of plasma cells.

Types

Symptomatic Waldenstrom macroglobulinemia

Symptomatic Waldenstrom macroglobulinemia causes symptoms or other health issues that call for treatment.

Smoldering Waldenstrom macroglobulinemia

Smoldering Waldenstrom macroglobulinemia also is called asymptomatic Waldenstrom macroglobulinemia. It means Waldenstrom macroglobulinemia is present but is not causing symptoms or organ damage that calls for treatment. People with smoldering Waldenstrom macroglobulinemia are watched closely instead of treated right away.

Symptoms

Waldenstrom macroglobulinemia worsens slowly. It might not cause symptoms for years.

When symptoms appear, they may include:

  • Extreme tiredness, also called fatigue.
  • Fever.
  • Soaking night sweats.
  • Weight loss that happens without trying.
  • Numbness or weakness in the arms or legs.
  • Swollen lymph nodes, which may feel like lumps under the skin. They happen most often in the neck, armpits and groin.
  • A feeling of pain or fullness under the ribs on the left side. This may be caused by an enlarged spleen.
  • Easy bruising.

Waldenstrom macroglobulinemia can make the blood too thick and slow its flow. This is called hyperviscosity syndrome. It can happen when too much IgM builds up in the blood. Symptoms may include:

  • Vision changes such as blurry vision, double vision or vision loss.
  • Headache.
  • Dizziness.
  • Ringing in the ears.
  • Sudden hearing loss.
  • Bleeding nose or gums.
  • Shortness of breath.
  • Confusion.
  • Memory loss.
  • Fainting.

When to see a doctor

Make an appointment with a healthcare professional if you have symptoms that worry you.

When to seek emergency care

Get emergency care right away for symptoms of hyperviscosity syndrome. Also get emergency care for unusual bleeding or bleeding from the nose that does not stop with simple pressure or packing.

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Causes

It's not clear what causes Waldenstrom macroglobulinemia. Cancer starts when cells have changes in their DNA. DNA gives cells instructions. Healthy cells grow and die in a controlled way. Cancer-related DNA changes can tell cells to grow too much and live too long. This can lead to a buildup of cancer cells.

In Waldenstrom macroglobulinemia, these changes happen in B cells. B cells, also called lymphoplasmacytic cells, are a type of white blood cell. The changed cells can build up in bone marrow and crowd out healthy blood cells. They also may build up in lymph nodes and the spleen.

Waldenstrom macroglobulinemia cells make a monoclonal protein called IgM. IgM can build up in the blood. This can make the blood too thick and slow its flow. This can affect organs and cause other issues.

Most gene changes in Waldenstrom macroglobulinemia cells happen randomly. They usually are not passed down from a parent. MYD88 is the most common gene change. CXCR4 is another common gene change. Family history can raise the risk of Waldenstrom macroglobulinemia. But there is no single inherited gene that explains most family cases.

Risk factors

Factors that can raise the risk of Waldenstrom macroglobulinemia include:

  • Older age and sex. Waldenstrom macroglobulinemia can happen at any age. But it's most common in adults 70 and older. It also occurs more often in men than in women.
  • Race and ethnicity. Waldenstrom macroglobulinemia is diagnosed more often in white people than in people of other races.
  • A family history of Waldenstrom macroglobulinemia or related conditions affecting B cells. Having a first-degree relative, such as a parent, sibling or child, with Waldenstrom macroglobulinemia or a related condition affecting B cells may raise the risk.
  • A history of autoimmune diseases. People with a history of autoimmune diseases have a higher risk of Waldenstrom macroglobulinemia.
  • Monoclonal gammopathy of undetermined significance (MGUS). Monoclonal gammopathy of undetermined significance (MGUS) is a condition in which a monoclonal protein is found in the blood. There are different types of MGUS. In IgM MGUS, the monoclonal protein is IgM.
  • IgM MGUS is not cancer. It can raise the risk of some blood cancers, including Waldenstrom macroglobulinemia. However, most people with IgM MGUS do not develop Waldenstrom macroglobulinemia.

Complications

Complications of Waldenstrom macroglobulinemia may include:

  • Hyperviscosity syndrome. Some people with Waldenstrom macroglobulinemia develop hyperviscosity syndrome. This means extra IgM proteins in the blood make the blood too thick so it flows more slowly. Hyperviscosity syndrome can be life-threatening.
  • Bing-Neel syndrome. Bing-Neel syndrome happens when Waldenstrom macroglobulinemia cells affect the brain or spinal cord. Symptoms of Bing-Neel syndrome include headaches, seizures, weakness, numbness, tingling or other nerve symptoms. The syndrome can happen when Waldenstrom macroglobulinemia is first found or later, even when it seems to be controlled.
  • Bing-Neel syndrome is rare. It affects about 1% of people with Waldenstrom macroglobulinemia. An MRI scan of the brain and spine and a test of spinal fluid can help diagnose Bing-Neel syndrome.

  • Low blood cell counts. As Waldenstrom macroglobulinemia gets worse, it may cause low red blood cells, called anemia, and other low blood counts, such as low platelets. This can happen when cancer cells build up in the bone marrow and crowd out healthy blood cells.
  • Larger than usual lymph nodes and spleen. Waldenstrom macroglobulinemia cells can build up in these organs and cause them to become enlarged.
  • A switch to a more aggressive form of cancer. Some people with Waldenstrom macroglobulinemia can develop a more aggressive form of cancer called aggressive lymphoma.
  • Secondary cancers. People with Waldenstrom macroglobulinemia are at a higher risk of developing other cancers. These may include thyroid cancer, kidney cancer and melanoma.
  • Amyloidosis. Amyloidosis (am-uh-loi-DO-sis) is a rare condition that happens when a protein called amyloid builds up in organs. This amyloid buildup can affect the way organs work. Amyloidosis may affect the heart, kidneys and nervous system.

Oct. 17, 2026

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