Lymphoplasmacytic lymphoma (LPL) or Waldenström’s macroglobulinemia (WM) is a type of non-Hodgkin lymphoma.

It is a cancer that occurs when there is an abnormal growth in antibodies derived from white blood cells called B lymphocytes.

White blood cells are building blocks of the immune system. White blood cells called B lymphocytes help protect against infection by developing into plasma cells and making antibodies.

Antibodies can identify bacteria and viruses that invade the body and attach to them. This helps the immune system rid the body of pathogens.

When LPL develops, the body produces abnormal antibodies. The most common is called IgM paraprotein. When this occurs, doctors refer to LPL as Waldenström’s macroglobulinemia (WM).

In this article, we will examine the symptoms, causes, diagnosis, treatment, and outlook for LPL, as well as when to contact a doctor.

An image of a blood test that helps diagnose LPL.Share on Pinterest
Kubra Cavus/Getty Images

LPL starts in B cells in the immune system. It occurs when the body overproduces an antibody called IgM, which can lead to symptoms such as bleeding, vision problems, and nervous system problems.

Cancer cells infiltrate the bone marrow and prevent other cells from doing their work. On a blood test, this can show up as low red blood cell count, known as anemia.

People with this condition will feel tired and weak. It may also cause low white cell counts, which prevent the body from fighting off infections. Blood platelets can drop, which results in easy bruising and bleeding.

It is a rare disease that most commonly appears in older, white males.

LPL is a slow-growing type of cancer that may take years to cause symptoms. Some people receive diagnosis following a routine blood test.

If a person has B cells in their bone marrow, the bone marrow may not be able to produce the number of normal blood cells it needs. This can lead to:

  • anemia, a shortage of red blood cells, leading to fatigue, weakness, and breathlessness
  • neutropenia, a shortage of neutrophils, a type of white blood cell, leading to risk of infection
  • thrombocytopenia, a shortage of platelets that help blood clot, leading to easy bruising or bleeding

Other symptoms of lymphoma that people with LPL may experience include:

  • swollen lymph nodes
  • swollen spleen, causing abdominal discomfort
  • fever
  • night sweats
  • weight loss

Very rarely, people might experience:

If IgM antibody builds up in the blood, it can make the blood thick. This is called hyperviscosity and can cause symptoms that include:

LPL develops from the abnormal growth of white blood cells called B lymphocytes. These cells grow into plasma cells, which make antibodies. When LPL occurs, the body produces large amounts of abnormal antibodies.

A condition called monoclonal gammopathy of undetermined significance (MGUS) causes the body to make large amounts of one specific antibody. On its own, MGUS does not cause issues, but annually about 1–2% of people with MGUS develop related cancers, such as LPL.

Other risk factors that increase an individual’s chances of developing LPL include:

  • Age: LPL is rare in people under the age of 50.
  • Race: LPL is more common among white people.
  • Sex: Males are more likely to develop LPL.
  • Heredity: About one in five people who develop the disease have a close relative who also had it or who had a related B-cell disease.
  • Hepatitis C: Some studies suggest people with chronic hepatitis C infection might be at higher risk for LPL.
  • Autoimmune disease: People with certain autoimmune diseases, such as Sjögren’s syndrome, may be at higher risk.

A person often receives an LPL diagnosis when getting regular blood work done at a checkup. Because it is a slow-growing cancer, it may not produce symptoms early on. However, there are exceptions.

If symptoms suggest LPL is present, a doctor will run tests to look for abnormal proteins in the blood and abnormal cells in the bone marrow.

The doctor will also perform a physical examination, looking for changes in lymph nodes, the eyes, spleen, and liver. They may also check for infections that could cause many of the same symptoms as LPL.

A doctor may order lab tests, a biopsy, or imaging tests to further examine areas of concern or check for the presence of cancerous cells.

If tests indicate LPL, doctors will assign it a stage according to how much cancer is present.

For cancer, doctors typically factor in the advancement of the disease during staging. However, with LPL, doctors do not. This is because research suggests this information is irrelevant to overall outcomes or designing a course of treatment.

Doctors will take into account factors such as age, blood cell counts, the number of abnormal antibodies present, and the level of a protein called beta-2 microglobulin.

For patients with WM, which are the vast majority of LPL patients, a system called the International Prognostic Scoring System for Waldenström Macroglobulinemia (ISSWM) helps with staging.

ISSWM takes into account factors such as platelet count, age, and blood hemoglobin level. Each factor is worth one point. The total scores refer to one of three risk groups:

  • Low risk: Includes patients 65 years or younger with no more than one point.
  • Intermediate risk: Includes patients older than 65 with two or fewer points or those younger than 65 with two points.
  • High risk: Includes people of any age with at least three points.

Treatment for LPL is similar to many other cancers. It may include:

Because LPL is a slow-growing cancer, some doctors may advise watchful waiting early in treatment to see how it develops and begin treatment when symptoms start.

LPL is a low-grade type of lymphoma that may grow slowly for many years. Treatment is generally effective, but the disease has a high probability of relapse, so treatment may need to be resumed. If there is a relapse, people are often initially put on active monitoring to see how the disease progresses.

For people with WM, the overall five-year survival rate is about 78%. The National Cancer Institute bases this rate on people who received a diagnosis between 2001 and 2010.

Someone with LPL may never have any symptoms and will only discover the cancer when they get a blood test during a routine appointment.

If a person notices any of the symptoms of LPL, they should consider contacting a doctor for an evaluation.

LPL is a rare type of slow-growing lymphoma that develops when there is an overproduction of certain types of antibodies related to B cells. It most often occurs in white males ages 60 years and older.

Scientists are not certain what causes LPL, but people with some autoimmune diseases or infections may have a higher risk of developing it.

Treatment may include watchful waiting or standard cancer therapies, including chemotherapy, radiation, or stem cell therapy.