WaldenströM's Macroglobu... Health Article

Definition

Waldenström's macroglobulinemia is a rare, chronic cancer of the immune system that is characterized by hyperviscosity, or thickening, of the blood.

Description

Waldenström's (Waldenstrom, Waldenstroem's) macroglobulinemia (WM) is a lymphoma, or cancer of the lymphatic system. It was first identified in 1944, by the Swedish physician Jan Gosta Waldenström, in patients who had a thickening of the serum, or liquid part, of the blood. Their blood serum contained a great deal of a very large molecule called a globulin. Thus, the disorder is called macroglobulinemia.

Lymphomas are cancers that originate in tissues of the lymphatic system. All lymphomas other than Hodgkin's disease, including WM, are known collectively as non-Hodgkin's lymphomas. There are 13 major types of non-Hodgkin's lymphomas, and others that are very rare. Other names that are sometimes used for WM include: lymphoplasmacytic lymphoma, lymphoplasmacytic leukemia, macroglobulinemia of Waldenström, primary macroglobulinemia, Waldenström's syndrome, Waldenström's purpura, or hyperglobulinemic purpura. Purpura refers to purple spots on the skin, resulting from the frequent bleeding and bruising that can be a symptom of WM.

WM is classified as a low-grade or indolent form of lymphoma because it is a slow-growing cancer that produces fewer symptoms than other types of lymphomas. WM most often affects males over the age of 65. Frequently, this disease produces no symptoms and does not require treatment. It has not been studied as extensively as other types of lymphoma.

The lymphatic system

The lymphatic system is part of the body's immune system, for fighting disease, and part of the blood-producing system. It includes the lymph vessels and nodes, and the spleen, bone marrow, and thymus. The narrow lymphatic vessels carry lymphatic fluid from throughout the body. The lymph nodes are small, pea-shaped organs that filter the lymphatic fluid and trap foreign substances, including viruses, bacteria, and cancer cells. The spleen, in the upper left abdomen, removes old cells and debris from the blood. The bone marrow, the spongy tissue inside the bones, produces new blood cells.

B lymphocytes or B cells are white blood cells that recognize disease-causing organisms. They circulate throughout the body in the blood and lymphatic fluid. Each B lymphocyte recognizes a specific foreign substance, or antigen. When it encounters its specific antigen, the B cell begins to divide and multiply, producing large numbers of identical (monoclonal), mature plasma cells. These plasma cells produce large amounts of antibody that are specific for the antigen. Antibodies are large proteins called immunoglobulins (Igs) that bind to and remove the specific antigen.

A type of Ig, called IgM, is part of the early immune response. The IgM molecules form clusters in the bloodstream. When these IgM clusters encounter their specific antigen, usually a bacterium, they cover it so that it can be destroyed by other immune system cells.

Plasma cell neoplasm

WM is a type of plasma cell neoplasm or B-cell lymphoma. These are lymphomas in which certain plasma cells become abnormal, or cancerous, and begin to grow uncontrollably. In WM, the cancerous plasma cells overproduce large amounts of identical (monoclonal) IgM antibody. This IgM also is called M protein, for monoclonal or myeloma protein.

Macroglobulinemia refers to the accumulation of this M protein in the serum of the blood. This large amount of M protein can cause the blood to thicken, causing hyperviscosity. The malignant plasma cells of some WM patients also produce and secrete partial immunoglobulins called light chains, or Bence-Jones proteins. The malignant plasma cells can invade various tissues, including the bone marrow, lymph nodes, and spleen, causing these tissues to swell.

WM accounts for about 1-2% of non-Hodgkin's lymphomas. It is estimated that it may affect about five out of every 100,000 people. It usually affects people over the age of 50, and most often develops after age65. It is more common in men than in women. In the United States, WM is more common among Caucasians than among African Americans. The disease can run in families.

Causes and symptoms

The cause of WM is not known.

Many individuals with WM have no symptoms of the disease. This is known as asymptomatic macroglobulinemia. When symptoms of WM are present, they may vary greatly from one individual to the next.