Waldenstrom's Disease

Written by Darla Burke | Published on August 20, 2012
Medically Reviewed by George Krucik, MD

What Is Waldenström’s Disease?

Your immune system produces different cells that protect your body against infection. One such cell is the B lymphocyte (also know simply as a “B cell”). These cells are made in the bone marrow, and migrate and mature in the lymph nodes and spleen. They can become plasma cells that are responsible for releasing an antibody known as immunoglobulin M or IgM. Antibodies are used by your body to attack invading diseases.

In rare cases, your body may begin to produce too much IgM. When this happens, your blood will become thicker (a symptom known as “hyperviscosity”), making it difficult for all of your organs and tissues to function properly. This condition is known as Waldenström’s disease. It is technically a type of cancer.

Waldenström’s disease is a rare cancer. The National Cancer Institute (NCI) reports that there are about 1,500 cases of Waldenström’s disease diagnosed each year in the U.S. (NCI, 2012). The disease is a non-Hodgkin lymphoma that is indolent (slow-growing). Waldenström’s disease is also known as:

  • Waldenström’s macroglobulinemia (WM)
  • lymphoplasmacytic lymphoma
  • macroglobulinemia—primary

What Are the Causes of Waldenström’s Disease?

Waldenström’s disease develops when your body produces too much IgM. Doctors do not know what causes this to happen.

The condition is more common in families. This suggests that Waldenström’s disease may be hereditary.

What Are the Symptoms of Waldenström’s Disease?

The symptoms of Waldenström’s disease will vary based on the severity of your condition. In some instances, people with this condition have no symptoms. The most common symptoms of this disease are:

  • weakness
  • fatigue
  • bleeding from the gums or nose
  • weight loss
  • bruises
  • skin lesions
  • skin discoloration
  • swollen glands

If the amount of IgM in your body becomes severely high, you may experience additional symptoms. These symptoms frequently occur as a result of hyperviscosity and include:

  • vision changes including blurry vision and vision loss
  • headache
  • dizziness (vertigo)
  • changes in mental status

How Is Waldenström’s Disease Diagnosed?

To diagnose this disease, your doctor will start by performing a physical exam and ask you about your health history. During your exam, your doctor may note that your spleen, liver, or lymph nodes are swollen.

If you have symptoms of Waldenström’s disease your doctor may also order additional tests to confirm your diagnosis. These tests may include the following:

  • blood tests to determine your level of IgM and to evaluate the thickness of your blood
  • bone marrow biopsy
  • CT scan of bones or soft tissue
  • X-ray of bones or soft tissue

CT scan and X-ray of the bones and soft tissues are used to differentiate between Waldenström’s disease and a similar type of cancer called multiple myeloma.

How Is Waldenström’s Disease Treated?

There is no cure for Waldenström’s disease. However, treatment can be effective for controlling your symptoms. Treatment for Waldenström’s disease will depend on the severity of your symptoms. If you have Waldenström’s disease but have no symptoms of the disorder, your doctor may not recommend any treatment. You may not require treatment until you develop symptoms. This may take several years.

If you have symptoms of the disease, there are several different treatments that your doctor may recommend including:

Chemotherapy

Chemotherapy is a medicine that destroys fast growing cells in the body. This treatment can be provided through your veins (intravenously) or as a pill. Chemotherapy in Waldenstrom’s disease is designed to attack the abnormal cells producing the excess IgM.

Plasmapheresis

Plasmapheresis or plasma exchange is a procedure in which excess proteins (IgM immunoglobulins) in the plasma are removed from the blood, and the remaining plasma is returned to the body.

Biotherapy

Biotherapy or biological therapy is used to boost the immune system’s ability to fight cancer. It can be used in conjunction with chemotherapy.

Surgery

In some instances, your doctor may recommend surgery to remove the spleen (splenectomy). Patients that undergo this procedure may be able to reduce or eliminate their symptoms for many years. However, patients that have a splenectomy often experience remission in which the symptoms of the disease return.

Clinical Trials

Following your diagnosis, you should also ask your doctor about clinical trials for new medications and procedures to treat Waldenström’s disease. 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 combat the disease.

What Is the Long-Term Outlook for a Patient With This Condition?

If you are diagnosed with Waldenström’s disease, your prognosis will depend on the progression of your disease. In some patients the disease will progress more slowly. These patients will have a longer survival time compared with those whose disease progresses more quickly. The average survival time for patients with this disorder is 6.5 years. Some patients will live more than 10 years following their diagnosis.

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