multiple myeloma

Plasmocytoma: description

A plasmocytoma is a special form of blood cancer in which so-called plasma cells in the bone marrow multiply uncontrollably. Other names for the plasmocytoma are “Kahler’s disease” and “multiple myeloma”. Strictly speaking, a multiple myeloma refers to a diffuse distribution of the proliferating plasma cells in the bone marrow. The plasmocytoma, on the other hand, has a localized (solitary) plasma cell proliferation.

The classification system for blood cancers is complicated. The plasmocytoma is classified in the group of lymphomas (so-called B-cell non-Hodgkin’s lymphoma).

Degenerate plasma cells

In the bone marrow, red and white blood cells are produced. While the red blood cells (erythrocytes) are responsible for the transport of oxygen in the body, the white blood cells (leukocytes) are formed in the bone marrow for immune defense. There are several subgroups of leukocytes, such as granulocytes, T or B cells. Plasma cells are the most mature stage of B cells and are responsible for the production of antibodies.

In a plasmocytoma plasma cells proliferate in the bone marrow uncontrolled. In addition, they produce large quantities of abnormal proteins: complete or incomplete altered antibodies of a single type (monoclonal antibodies) called paraproteins. As a result, the immune system is weakened in a plasmocytoma, making the patient much more susceptible to infection. Over time, the degenerate plasma cells displace more and more healthy cells in the bone marrow, which can cause various symptoms.

Plasmocytoma: frequency

About every fourth to fifth of 100,000 people in Germany suffer from a plasmocytoma. Men are affected more frequently than women. The average age of onset of the plasmocytoma is over 45 years.

Plasmocytoma: symptoms

At the beginning of the cancer in the bone marrow usually causes no complaints. Only later can multiple myeloma cause symptoms of various kinds:

back pain

Bone pain is the first symptom of a plasmocytoma. Particularly often those affected complain of back pain. By Plasmozytom bone tissue is degraded (often in the area of ​​the spinal column). The risk of broken bones (fractures) is therefore increased.

Displacement of red blood cells

In a plasmocytoma, altered plasma cells proliferate in the bone marrow. As a result, other important blood cells are displaced in their growth. As a result, too little red blood cells are formed, and there is anemia (anemia). The symptoms of anemia are due to the resulting lack of oxygen in the tissues: pale skin, feeling weak, dizziness and tiredness.

Antibody deficiency

If the healthy white blood cells are also displaced, not enough intact antibodies can be produced. The immune system is thus weakened by the plasmocytoma, and it is easier to get infections with bacteria or viruses.

kidney damage

Part of the paraproteins produced by the plasmocytoma is excreted by the kidneys. However, these so-called Bence Jones proteins can also accumulate in the kidney tissue and damage it. Some patients subsequently report foaming urine.

Small skin bleeding

The formation of platelets (platelets) is also affected by a Plasmozytom. The platelets are usually responsible for blood clotting. As a result, small pinhead-sized bleeding in the skin (petechiae) is more common.

Plasmocytoma: causes and risk factors

The starting point for a plasmocytoma is formed by a degenerate plasma cell, which increases exponentially. Plasma cells are among the B lymphocytes, a subset of white blood cells. Their most important task is the production of antibodies. However, degenerate plasma cells produce altered antibodies (paraproteins).

How degeneration of plasma cells occurs in the plasmocytoma has not yet been fully elucidated. Scientists were able to detect a genetic change in 15 percent of those affected. Chromosomes 13 and 14 showed more defects. The influence of ionizing radiation and pesticides on the formation of plasmocytoma is currently being investigated as well.

The plasmocytoma causes the formation of many new blood vessels in the bone marrow so that it is optimally supplied with nutrients and can grow.

Plasmocytoma: examinations and diagnosis

For any symptoms that may indicate a plasmocytoma, patients should seek medical attention. He can determine with various investigations, whether actually a multiple myeloma is present.

Blood and urine examination

The blood test is a quick and convenient way to get first clues to a plasmocytoma. The degenerate antibodies are detectable in the blood based on an elevated protein level. The protein level correlates with the plasmocytoma activity: the more degenerate proteins are present, the more advanced is the plasmocytoma.

A blood count shows the proportion of different blood cells. This shows how strongly the plasmocytoma has already displaced healthy cells and whether there is already anemia. In addition, blood counts can help detect altered kidney values ​​when Bence Jones proteins damage kidney tissue and impair kidney function.

Bone infestation by the plasmocytoma results in increased calcium levels in the blood picture: The bone consists to a large extent of calcium. If the plasmocytoma accelerates the bone breakdown, the released calcium is distributed in the blood and can be measured.

The Bence Jones proteins, also degenerate proteins from the plasma cells, can be detected in the urine of patients.

Bone marrow

If you suspect a plasmocytoma, you can perform a bone marrow puncture. As a rule, the iliac crest is pierced with a needle in order to extract some bone marrow. Subsequently, the bone marrow sample is examined under the microscope. In healthy individuals, plasma cells usually account for a maximum of five percent. In contrast, plasmocytoma patients often have values ​​of more than ten percent. The degenerate cells can be further examined by determining the rate of division or by searching for possible chromosomal changes.

Imaging procedures

If the plasmocytoma has led to bone loss, this can be demonstrated in the X-ray image. You can see small holes (osteolytic foci) in the skull, in the ribs, in the vertebral bodies or in the pelvic bone.

Computed tomography (CT) is another imaging test method for plasmocytoma diagnostics. It shows even more clearly how the skeletal system has already been affected by the plasmocytoma.

If there is a suspicion that the spinal cord has already been damaged by the disintegration of the vertebral bodies, an additional magnetic resonance imaging (MRI) can be performed.

Plasmocytoma: treatment

Plasmacytoma therapy depends on the stage of the disease. Three stages of disease are differentiated: the higher the stage, the more malignant the plasmocytoma. A precise classification is of great importance to be able to create an adequate treatment plan.

If there are no complaints and if the stage I is a plasmocytoma, the patient is closely examined and monitored (“watch and wait”).

Multiple myeloma: chemotherapy and stem cell transplantation

Chemotherapy is started as soon as the plasmocytoma spreads. The aim of the therapy is to prevent further spreading of the degenerated plasma cells.

In advanced stage II and III plasmocytoma, high dose chemotherapy is used. This greatly weakens the patient’s immune system. Therefore, if possible, a so-called autologous stem cell transplantation is carried out: The patient’s own stem cells – taken before chemotherapy – administered again, so that the formation of immune and blood cells quickly gets going again. Patients under the age of 75 are the current standard procedure. Patients older than 75 years or patients in very poor general condition are not eligible for high-dose chemotherapy.

Multiple myeloma: therapy with other drugs

Not every patient can be treated with high dose chemotherapy and subsequent stem cell transplantation. In addition, this combined treatment does not work in some patients, resulting in relapses. In both cases, the administration of other drugs may be useful.

high-dose glucocorticoids (Dexamethasone, prednisolone) can cause a rapid reduction in tumor mass.

The active substance bortezomib is a so-called proteasome inhibitor. It causes cancer cells to die, and prevents the formation of new blood vessels. Without proper blood supply, the plasmocytoma can not continue to grow.

The two active ingredients thalidomide and lenalidomide belong to the group of IMiD (immunomodulating drugs, immunomodulating drugs). They also prevent the ingrowth of new blood vessels in the bone marrow and trigger the cell death of cancer cells. In addition, they inhibit the release of inflammatory substances.

The substances are used and dosed individually. There may also be other drugs in the combination therapy. In order to avoid relapse, a combination of lenalidomide, thalidomide and bortezomib is often used.

Supportive therapy

The plasmocytoma can be extremely painful, especially in the case of bone involvement. The pain can be treated with radiation therapy in addition to chemotherapy. In the process, individual tumor sites are irradiated in order to inhibit cell growth.

In addition, so-called bisphosphonates will be added. Bisphosphonates inhibit bone resorption and have a stabilizing effect on the bones.

Since the immune defense is weakened by the plasmocytoma, special attention should be paid to the protection against infections. A flu vaccine or a vaccine against pneumococci is very useful for many people affected. In addition, the patient should especially in the flu season (autumn, winter) wash his hands more often. An increased risk of infection can be found in public transport, kindergartens and schools.

Plasmocytoma: disease course and prognosis

The plasmocytoma can vary widely in its course and prognosis. A role is played by the stage of the disease, the age of the patient as well as possible comorbidities. The treatment goal is always the extension of the lifetime with maximum possible quality of life.

Multiple myeloma: prognosis

Complete healing is only possible in very few cases. However, the plasmocytoma prognosis is already significantly better than before due to the current therapeutic possibilities. Before chemotherapy was the standard procedure, the mean survival time was about one year. With chemotherapy she is today at about five years. As unfavorable prognostic factors are:

• a change in the chromosome 13
• a high stage of plasmocytoma
• a great age

Generally, however, a multiple myeloma applies: Life expectancy in individual cases can not be predicted accurately. Some patients die within a few months, other patients still live after ten years.

Multiple myeloma: terminal stage

In the final stage, the plasmocytoma is already very large. Often patients die because of that multiple myeloma no longer enough blood cells are formed in the bone marrow.