Waldenström’s disease is a rare type of blood cancer that causes weakness, tiredness, and persistent infections. There is currently no cure for Waldenström’s disease, but there are treatments for the symptoms.

Waldenström’s disease is a type of cancer of the blood that causes white blood cells to grow unusually.

Another name for Waldenström’s disease is Waldenström macroglobulinemia (WM). WM is a slow-growing subcategory of non-Hodgkin lymphoma.

In this article, learn more about WM, including its treatments and the outlook for people with the condition.

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WM occurs when B lymphocytes, which are a type of white blood cell, grow unusually. Healthy cells typically divide and replicate as part of their natural life cycle, but these cells grow uncontrollably in people with WM.

According to the National Organization for Rare Disorders, WM affects around 1 in 3.4 million men and about half as many women in the United States. According to the American Cancer Society (ACS), around 1,000–1,500 people receive a diagnosis of WM each year.

WM occurs more frequently with age. The ACS also states that the average age of diagnosis is 70 years. However, anyone can get WM, including people in their 20s.

When someone has a condition without any symptoms, a doctor may refer to them as asymptomatic. Around 1 in every 4 people with WM are asymptomatic at the time of their diagnosis. So, a doctor may identify the condition using routine blood tests.

People with symptoms may experience weakness, extreme tiredness, and frequent or long lasting infections. Some other common WM symptoms include:

Some less common symptoms may include:

Some symptoms are the result of high immunoglobulin M (IgM) levels in the blood. This makes the blood thicken and move more slowly. Blood-thinning medications are unable to treat this type of thickening in the blood.

WM begins in B lymphocytes when they begin to grow out of control. Doctors refer to these uncontrollably growing cells as lymphoplasmacytoids. The cells produce an unusually large amount of IgM in the blood.

IgM is important for fighting off diseases, but having too much of it can cause hyperviscosity syndrome. This occurs when the blood becomes too thick to move as quickly as it should, causing symptoms such as:

Hyperviscosity syndrome can also cause severe symptoms, such as:

Scientists do not know exactly what causes WM, but some believe that genetic changes may be one possible cause.

According to the ACS, recent research suggests that 9 out of 10 occurrences of WM are due to a mutation in the MYD88 gene. This gene normally helps cells stay alive. Researchers think that this mutation could cause the gene to be permanently switched on, causing WM cells to live longer than they should.

Around 30–40% of people with WM have a mutation in the CXCR4 gene. There is some evidence to suggest that these people have more severe disease that is less responsive to treatment.

Several factors can increase the risk of WM, including:

  • Sex: WM seems to affect more men than women.
  • Age: WM is rare in people younger than 50 years.
  • Ethnicity: WM is more common in people with white American than African American backgrounds.
  • Heredity: Having a close relative with a related condition increases the risk of WM.
  • Hepatitis C: Some research suggests that having hepatitis C increases the risk of WM.
  • Autoimmune conditions: There is some evidence suggesting that autoimmune conditions can increase the risk of WM.

A doctor needs to conduct a blood test to check IgM levels when diagnosing WM. They may also order a bone marrow biopsy, CT scans, and X-rays.

Doctors also need to determine the stage of the condition. This can tell them how severe the cancer is and what treatment options may be best.

However, the stage appears to have little impact on a person’s outlook or the best treatment options for WM. This is because other factors are more important to disease outcomes, including:

  • age
  • blood cell counts
  • IgM levels in the blood
  • beta-2 microglobulin protein levels

In people with WM who do not experience symptoms, a doctor may choose to defer treatment until symptoms appear.

The first line of treatment may involve rituximab, which is a monoclonal antibody that depletes B cells so that the body produces less IgM.

Some other types of treatment for WM may include:

  • Chemotherapy: Chemotherapy treatment may include the drugs cyclophosphamide or bendamustine.
  • Radiation therapy: With radiation therapy, doctors use rays to kill or shrink the cancer cells.
  • Targeted drug therapy: Targeted therapy only attacks the affected cells rather than the whole body, which is what chemotherapy does. These drugs include bortezimib, ibrutinib, and zanubrutinib.
  • Stem cell therapy: This treatment may cause fewer side effects than chemotherapy or radiation therapy. It is not a common treatment for WM, but doctors may recommend it for younger people. Doctors usually carry out stem cell transplants by first taking blood.
  • Plasmapheresis: This treatment may be useful in people with hyperviscosity syndrome. The procedure involves filtering a person’s blood through a machine, resulting in less IgM, which thickens the blood.

The International Prognostic Scoring System for Waldenstrom Macroglobulinemia assigns a point for each of the following factors:

  • a blood hemoglobin level of 11.5 grams per deciliter (g/dl) or under
  • a platelet count of 100,000 per microliter or under
  • a beta-2 microglobulin level of more than 3 milligrams per dl
  • a monoclonal IgM level of more than 7 g/dl

The resulting scores divide people into three categories:

  • Low risk: This is anyone under 65 years with 1 point or fewer.
  • Intermediate risk: This is anyone with 2 points and those older than 65 years.
  • High risk: This is anyone with 3 or more points.

According to a 2015 research paper, the median survival rate for people with WM ranges from 5 to almost 11 years. The study suggests that the causes of death are typically due to disease progression and other age-related reasons.

WM is a rare form of blood cancer that causes the uncontrollable growth of white blood cells. There is currently no cure for WM, but doctors can manage the symptoms with appropriate treatment.

Anyone who experiences any symptoms of WM should speak with a doctor right away. The symptoms may be due to another condition, which the doctor can rule out.