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Macroglobulinemia

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Waldenström's disease
From Gale Encyclopedia of Medicine, 4/6/01 by J. Ricker Polsdorfer

Definition

Waldenström's disease, also known as macroglobulinemia, is a leukemia-like disorder characterized by the buildup of large antibodies (IgM) in the blood.

Description

The bone marrow, spleen and certain other tissues are involved in producing the cells that circulate throughout the body. In addition to red blood cells, which stay in the blood vessels, they produce the body's immune defense system, which has both cellular and chemical components. These immune components roam through tissues and do not confine themselves to the blood vessels. The cellular components of immunogenic agents are mostly white blood cells. The chemical components, proteins called globulins, circulate by themselves in the blood after being made inside other cells called plasma cells. In Waldenström's disease, the plasma cells over produce large antibodies called macroglobulins (IgM).

Causes & symptoms

Waldenström's disease is a rare disorder that commonly occurs in men around 65 years of age. Its cause is unknown. Due to the buildup of large antibodies in the blood, the blood thickens and its circulation slows down. The patient feels weak and tired. There can be temporary paralysis. Infections are more common and recurrent. The thickened blood may affect the brain and nervous system, and the patient may feel dizzy, have funny feelings all over his body, get headaches, and have disturbed vision. Impaired circulation in the fingers, toes, and nose is evident.

Some patients have particular difficulty with circulation to their hands and feet during cold weather, because the globulins get even thicker. This condition is called cryoglobulinemia. Fingers and toes may turn white and ache, producing Raynaud's disease (A disease characterized by the suseptibility of fingers and toes to cold. Extremities are white or patchy red and white, cold, and painful).

Internal organs are stressed. Liver, spleen, and lymph nodes may enlarge. Heart conditions are aggravated. Abnormal bleeding may occur from the nose, mouth, and intestinal tract.

Diagnosis

Blood tests will indicate abnormal blood cell counts. Other tests may detect cryoglobulins. Immunoelectrophoresis (separation of proteins in the blood by solidifying them with electromotive force in order to identify various proteins and their counts) will detect the buildup of macroglobulins in the blood. A bone marrow biopsy may show elevated levels of lymphocytes and plasma cells.

Treatment

Waldenström's disease is presently incurable, however there are treatments that can relieve symptoms and retard the disease's development. Thinning the blood by passing it through a special filtering device relieves most of the symptoms. This process is called plasmapheresis. Cancer chemotherapy helps slow the abnormal development of plasma cells, but doesn't cure the disorder.

Prognosis

Many people survive five years or more, even without treatment.

Key Terms

Cryoglobulinemia
Disorder marked by an elevated level of cryoglobulins in the blood. Cryoglobulins solidify when exposed to cold and dissolve when warmed.
Immunoelectrophoresis
Separation of proteins in the blood by solidifying them with electromotive force in order to identify various proteins and their counts.
Paralysis
Inability to move.
Plasmapheresis
Thinning the blood by passing it through a special filtering device.
Raynaud's disease
Disease characterized by the suseptibility of fingers and toes to cold. Extremities are white or patchy red and white, cold, and painful.

Further Reading

For Your Information

    Books

  • Kyle, Robert A. "Plasma cell disorders." In Cecil Textbook of Medicine. Edited by J. Claude Bennett and Fred Plum. Philadelphia: W. B. Saunders, 1996.
  • Longo, Dan. L. "Plasma cell disorders." In Harrison's Principles of Internal Medicine. Edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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