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Waldenstrom macroglobulinemia

Waldenstrom macroglobulinemia; Macroglobulinemia - primary; Lymphoplasmacytic lymphoma; Monoclonal macroglobulinemia

 

Waldenstrom macroglobulinemia (WM) is a cancer of the B lymphocytes (a type of white blood cell). WM is associated with the overproduction of proteins called IgM antibodies.

Causes

 

WM is a result of a condition called lymphoplasmacytic lymphoma. This is a cancer of the white blood cells, in which the B or T immune cells start dividing rapidly. The exact cause of too much production of the IgM antibody is unknown. Production of excess IgM causes the blood to become too thick. This is called hyperviscosity. It can make it harder for blood to flow through small blood vessels.

WM is very rare. Most people with this condition are over 65 years of age. It may also occur in younger people.

 

Symptoms

 

Symptoms of WM may include any of the following:

  • Bleeding of the gums and nosebleeds
  • Blurred or decreased vision
  • Bluish skin
  • Dizziness
  • Easy bruising of the skin
  • Fatigue
  • Flank pain
  • Headache
  • Mental status changes
  • Numbness, tingling, or burning pain in the hands, feet, fingers, toes, ears, or nose
  • Rash
  • Swollen glands
  • Unintentional weight loss
  • Vision loss in one eye

 

Exams and Tests

 

A physical examination may reveal a swollen spleen, liver, and lymph nodes. An eye exam may show enlarged veins in the retina or retinal bleeding (hemorrhages).

A CBC shows a low number of red blood cells and platelets. Blood chemistry shows evidence of kidney disease. A serum viscosity test can tell if the blood has become thick. Symptoms usually occur when the blood is 4 times thicker than normal.

A test called serum protein electrophoresis shows an increased level of the IgM antibody. Levels are often higher than 3 grams per deciliter (g/dL).

Bone lesions are very rare. If they are present, a bone marrow examination will show cells that look like both lymphocytes and plasma cells.

Additional tests that may be done include:

  • 24-hour urine protein
  • Total protein
  • Serum globulin electrophoresis
  • Immunofixation in urine
  • T (thymus derived) lymphocyte count

 

Treatment

 

The treatment aim is to decrease the symptoms and the risk of developing organ damage.

Plasmapheresis removes unwanted substances from the blood. In MW, it removes or reduces the high level of IgM. It also quickly controls the symptoms caused by blood thickening.

Medicines may include corticosteroids, a combination of chemotherapy medicines and the monoclonal antibody to B cells, rituximab.

People who have a low number of red or white blood cells or platelets may need transfusions or antibiotics.

 

Outlook (Prognosis)

 

The average survival is about 5 years. Some people live more than 10 years.

In some people, the disorder may produce few symptoms and progress slowly.

 

Possible Complications

 

Complications of MW may include:

  • Changes in mental function, possibly leading to coma
  • Heart failure
  • Gastrointestinal bleeding
  • Vision problems
  • Hives

 

When to Contact a Medical Professional

 

Call your health care provider if symptoms of this disorder develop.

 

 

References

National Cancer Institute. PDQ adult non-Hodgkin lymphoma treatment. Bethesda, MD. National Cancer Institute. Updated June 1, 2016. www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq. Accessed June 3, 2016.

Rajkumar SV. Plasma cell disorders. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 187.

Treon SP, Merlini G. Waldenström macroglobulinemia and lymphoplasmacytic lymphoma. In: Hoffman R, Benz EJ, Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders; 2013:chap 86.

 
  • Waldenstroms

    Waldenstroms - illustration

    Lymphocytoid appearing plasma cell nucleus similar to a lymphocyte and cytoplasm similar to a plasma cell.

    Waldenstroms

    illustration

  • Antibodies

    Antibodies - illustration

    Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

    Antibodies

    illustration

  • Hypertensive retinopathy

    Hypertensive retinopathy - illustration

    Damage to the retina from high blood pressure is called hypertensive retinopathy. It occurs as the existing high blood pressure causes changes to the microvasculature of the retina. Some of the first findings in the disease are flame hemorrhages and cotton wool spots. As hypertensive retinopathy progresses, hard exudates can appear around the macula along with swelling of the macula and the optic nerve, causing impairment of vision. In severe cases permanent damage to the optic nerve or macula can occur.

    Hypertensive retinopathy

    illustration

    • Waldenstroms

      Waldenstroms - illustration

      Lymphocytoid appearing plasma cell nucleus similar to a lymphocyte and cytoplasm similar to a plasma cell.

      Waldenstroms

      illustration

    • Antibodies

      Antibodies - illustration

      Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens.

      Antibodies

      illustration

    • Hypertensive retinopathy

      Hypertensive retinopathy - illustration

      Damage to the retina from high blood pressure is called hypertensive retinopathy. It occurs as the existing high blood pressure causes changes to the microvasculature of the retina. Some of the first findings in the disease are flame hemorrhages and cotton wool spots. As hypertensive retinopathy progresses, hard exudates can appear around the macula along with swelling of the macula and the optic nerve, causing impairment of vision. In severe cases permanent damage to the optic nerve or macula can occur.

      Hypertensive retinopathy

      illustration

    A Closer Look

     

      Tests for Waldenstrom macroglobulinemia

       

       

      Review Date: 5/14/2016

      Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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