Plasmacytoma Treatment Information

PLASMACYTOMA TREATMENT INFORMATION

What are "Plasma Cells"?

Plasma cells are "B" lymphocytes in their final stage of development, when they are actually producing antibodies to fight "germs" like bacteria, viruses, fungi or protozoan parasites. They are an integral part of the immune system . There are basi-cally two types of immunity, called "cellular" and "humoral" immunity. Lymphocytes are white blood cell (about 30% of adult's while blood cells) that are actively involved in both types of immunity. "Cellular" immunity means that the white blood cells directly kill foreign invading germs, such as by enveloping and digesting them, while the "humoral" type means that the invaders are coated by antibodies and then gradually enveloped and digested by other white blood cells. The invading germs have markers on their cell surfaces, called "antigens" which tell the immune system that they are foreign to the body. The two common types of lymphocytes are "T" and "B" cells. "T" cells specialize in recognizing germs and activating the immune system, while "B" cells produce specific antibodies against the antigens that the "T" cells have recognized. The "B" cells go through a number of stages of development, and the final one is that they are specifically programmed to become antibody producing factories.

The antibodies produced are specific to attack a particular kind of germ or foreign tissue. At this final stage of development, they are called"plasma cells".

What is a Plasmacytoma?

Plasmacytoma is basically a cancer of the plasma cells, meaning that they are multiplying out of control. The body normally keeps a tight control on it's cells division; cell division is controlled by the genetic material ("genes") inside each cell. Cells commonly divide rapidly in womb life, childhood and puberty, to grow the adult body. After this, most divide more slowly to replace cells lost through old-age or injury. Blood cells, which include red cells to carry oxygen, white cells to fight infection, and platelets for clotting, continue to divide rapidly in adults, for these cells are usually short-lived. For the immune system, a great quantity of specific antibody is only needed to fight a specific invader, since the antibody produced for each type of germ is different. Thus, only particular white blood cells will be needed in large quantity. For example, if a person has strep, there is no need to be producing massive quantities of antibody against tetanus! Interestingly, the immune system is partly regulated by a class of "T" lymphocytes, called "T-suppressor" cells, which block excessive production of unneeded antibodies. Thus, the "T" lymphocytes not only stimulate the immune system via the "T-Helper" cell, but they also calm it down through the "T-Suppressor" cells.A cancer of the the immune system starts in just one cell . There are particular cancers for "T" cells and "B" cells, including leukemias andlymphomas . A plasmacytoma is the particular name for uncontrolled division of the plasma cells. Since it starts in just one cell, all of the plasma cells produced should appear identical, and moreover, produce the same antibody, also called "immunoglobulin." These exact copies of the original plasma cell which first produced the effective antibody are called"clones." When this production is well controlled, it fights disease. When it is out of control, it gains the capacity to invade and destroy normal tissue; this is cancer. For plasma cell cancer, when it is found in just one place in the body (usually bone) it is called (solitary) plasmacytoma . When it is in multiple areas, it is called "multiple myeloma".

How common is Plasmacytoma?

There are about 12,000 new cases of "plasma cell neoplasms", which includes plasmacytoma and multiple myeloma, each year in the U.S.A. About 1,000 of these cases are"solitary plasmacytoma" each year, the remainder are multiple myeloma at diagnosis. The number of cases increases with age. 98% of patients are over 40 years old, and 60% of patients are males. The disease is more common in Black individuals than Whites. Multiple Myeloma causes about 7000 deaths per year. Concerning plasmacytomas, there are two basic types--"solitary plasmacytoma of bone" and"extramedullary plasmacytoma." Obviously, the plasmacytoma of bone starts in bone. In contrast, the "extramedullary" type starts in soft tissue like fat or muscle. These types have different characters. Generally, the "plasmacytoma of bone" is more likely to progress to multiple myeloma (>70% at 10 years) than "extramedullary plasmacytoma (20% at 10 years). Multiple plasmacytomas are multiple myeloma by definition.

What Causes or Increases the Risk for Plasmacytoma?

Like any cancer, the particular reason why one person gets a plasmacytoma and another does not remains unknown . However, several things have been noted to increase the risk for plasma cell neoplasms, called"risk factors"

1) Radiation Exposure-- About 5 times the normal expected number of plasma cell cancers were seen in the Japanese atomic bomb survivors, this was not noted, however, until 20 years after the war! Routine X-rays are a minimal risk.
2) Heredity - Close relatives of patients with plasma cell cancer have a higher risk of getting it than the general population. Men and Women get the disease in equal numbers.
3) Carcinogens are chemicals that increase the risk of cancers. They are suspected of causing a gene disturbance in multiple myeloma, leading to an uncontrolled growth of plasma cells.

**Cigarette smoking and alcohol intake do not appear to increase the risk.

Can Plasma Cell Cancer be Prevented?

At this time, besides being prudent about not exposing one's self to unnecessary radiation or carcinogens, there is no known way of preventing plasma cell cancers.

What are the Symptoms of Plasmacytoma?

This of course depends upon it's location, and whether it is in bone or the soft tissues. For "solitary plasmacytoma of bone" the most common symptom ispain in the local area, or a"pathologic fracture" from weakening of that area of bone. There can be compression of nerve roots in the spine from a growing tumor. Alternatively, there are often no symptoms ; the disease is detected on a routine X-ray for a physical exam or by a chiropractor. For "extramedullary plasmacytoma" the most common symptom is a fleshy swelling which continues to enlarge over several months. The and symptoms of multiple myeloma are several areas of bone pain and anemia (with paleness and weakness).

How is Plasmacytoma Diagnosed and Evaluated?

When a patient presents with symptoms suspicious for a plasma cell cancer, such as new bone pain, an X-ray may show "lytic" (clear) areas in the bone, which is where the hard outer shell ("cortex") of bone is being destroyed by tumor. The only way to absolutely diagnose any cancer is to get a "biopsy", that is a sample of it . This sample can be obtained using a needle to punch through the skin and into the involved bone. In plasmacytoma or multiple myeloma, it will show lots of "plasma" cells when examined under a microscope. Also a bone marrow biopsy will show excessive plasma cells, called"plasmacytosis", if the disease is widespread. It is important to note that other conditions than plasma cell cancers can cause plasmacytosis. These include drug reactions, rheumatoid arthritis and cirrhosis of the liver. However, in combination with bone lesions, it strongly suggests a plasma cell cancer.

A patients who has lytic (destructive) bone lesions without another evident cancer to explain them will have laboratory studies performed on their blood and urine looking for evidence of abnormal immunoglobulin production. Recall that these immunoglobulins are the antibodies that the plasma cells produce. The most common type is Immunoglobulin G, abbreviated "IgG", followed by immunoglobulins "A", "D" and "E". The doctor orders a blood test called an "immunoglobulin electrophoresis" to look and see if a particular immunoglobulin is being disproportionately produced. If it is, a "monoclonal spike" will be seen on the immunoglobulin electrophoresis, this simply means that something is being produced by a specific type of plasma cell, and may or may not indicate cancer. In fact, elderly people can develop a "monoclonal gammopathy of unknown significance" (MGUS for short) which is entirely without symptoms but occasionally progresses to multiple myeloma. When a plasma cell cancer is present, we expect to see characteristic bone lesions in addition to the "monoclonal spike". Quite simply, if just one lesion is seen, this is a solitary plasmacytoma, while if multiple areas of bone destruction are seen, this is multiple myeloma. In patients with a plasma cell cancer, 55% produce excessive IgG, 20% produce too much IgA, and only 1% produce too much IgD or IgE. The more advanced the disease the higher the production rates of these immunoglobulins. If the immunoglobulin level is less than 2.0 grams per 100 milliliters of blood, and the X-ray is normal, its probably MGUS. If, on the other hand, the IgG or IgA level is greater than 7.0 grams per 100 milliliters of blood, and the X-ray is abnormal, this is almost certainly a plasma cell cancer, even without doing a biopsy of the bone. The level of the immunoglobulin produced is related to the "myeloma cell mass", that is the number of cancerous plasma cells, and so tells how advanced the disease is. The abnormal immunoglobulin produced is also called the "M" protein, and monitoring it's level during treatment can show the progress of the therapy.

When a patient has excessive immunoglobulin production from an abnormal clone (subgroup) of plasma cells, it may also show up in the urine. Specifically, the "M-protein" is composed of both heavy and light "chains" of protein, and the "light" chains can be excreted by the kidney into the urine. There are only two types of light chains, "kappa" or"lambda", and a patient will have only one type in the urine. The amount of these light chains in the urine can tell us how advanced the disease is. Less than 4 grams of light chains in the total urine produced over 24 hours means limited disease, while greater than 12 grams is a marker for advanced disease.

When patients have excessive light chains in the urine, of either the kappa or lambda type, this is called"Bence-Jones Proteinuria". The classic Bence-Jones test was a simple assay of the protein in the urine. High Bence-Jones protein is very suspicious for a plasma cell cancer. Yet another marker of extent of plasma cell disease is a blood test called "serum beta-2 microglobulin" which indicates excessive production of immunoglobulins by an abnormal group ("clone") of plasma cells. This test also tells the effectiveness of therapy, if the beta-2 microglobulin level changes.

Other tests are standardly obtained to help distinguish between solitary plasma- cytoma and multiple myeloma, and give the extent of disease (called the"stage" ) for all plasma cells cancers.

It is crucial to get prompt diagnosis and proper treatment for a Plasmacytoma problem. Getting the best treatment for this disease can actually make the difference between life and death. Understanding your options will give you the peace of mind knowing that you have done everything possible to ensure a happy outcome.

CancerAnswers' material explains, in plain English, the definition, types, frequency, risk factors, symptoms, evaluation, historic and latest effective treatment for Plasmacytoma. We tell you everything you must know to make the right choices today to deal with a Plasmacytoma problem.

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