Plasmacytoma • Multiple myeloma is incurable

Many tumor foci in the bone marrow are characteristic of multiple myeloma. If there is only one focus of the disease, it is a solitary (isolated) plasmacytoma. Multiple myeloma is classified in the group of so-called non-Hodgkin lymphomas.

Plasma cells are B lymphocytes and are part of the white blood cells. They play a role in the specific immune defense. When they come into contact with pathogens or foreign substances, they develop antibodies against them. In multiple myeloma, the excessive accumulation of plasma cells in the bone marrow disrupts blood formation and weakens the immune system with an increased susceptibility to infection. When the bones break down, there is a risk of fractures and osteoporosis. The pathological plasma cell centers also increasingly produce antibodies and proteins that damage other organs such as the kidneys or the brain as the disease progresses.

Plasmacytoma Stages: How Multiple Myeloma Progresses

Several classifications are used to estimate the stage of the disease and the expected course of the disease.

International staging system

Plasmacytoma staging according to Durie and Salmon

Frequency and prognosis of plasmacytoma (multiple myeloma)

In Germany, around six to seven out of 100,000 residents develop multiple myeloma every year. This makes it among the 20 most common types of cancer in Germany. The incidence increases with age, the average age of onset is 71 years.

The average Prognosis (life expectancy) with plasmacytoma is six years from diagnosis, but varies between a few and over ten years. The form of cancer is not curable yet.

Symptoms of plasmacytoma

The symptoms of plasmacytoma are very diverse and range from the absence of any symptoms to severe organ damage.

Usually, multiple myeloma begins without symptoms and progresses gradually over a period of a few weeks to months or even years. Uncharacteristic general complaints such as decreased performance, weakness, tiredness, loss of appetite, feeling thirsty, weight loss, night sweats and a slight fever are increasingly appearing.

The most common PlasmacytomaSymptoms at the time of diagnosis are:

• Bone pain (almost two thirds of all patients)
• Symptoms as a result of anemia: insufficient physical performance, severe shortness of breath during exertion, pale skin (half of all patients)

Less often or in a more advanced stage:

• Neurological disorders as a result of damage to the spine and entrapment of nerves, especially in the chest and lumbar area (back pain, numbness in the legs)

• painful broken bones as a result of the bone damage

• Polyneuropathy: Sensory disturbances and abnormal sensations above all
  in the legs

• Kidney dysfunction: heavily foaming urine, frequent urge to urinate
  with particularly large amounts of urine or the drying up of urine production (uremia)

• increased susceptibility to bacterial infections, for example purulent bronchitis, pneumonia, inflammation of the sinuses, urinary tract infections

An emergency is:

• Hypercalcemic crisis with excessive calcium concentration in the blood: nausea, vomiting, increased urination, fluid loss and dehydration (dehydration), neuropsychiatric disorders such as tiredness, slowing down, muscle weakness, drowsiness and even coma

• Hyperviscosity syndrome (disease-related change in the flow properties of the blood as a result of the increased antibodies and proteins that lead to viscous blood): abnormally increased bleeding tendency, cardiovascular disorders and neurological symptoms such as dizziness, concentration disorders, confusion or visual disturbances.

Causes of Multiple Myeloma

The causes of the plasmacytoma have not yet been conclusively researched. It has been shown that people of African descent are at higher risk for multiple myeloma. In addition, the disease occurs more frequently in some families. Scientists have shown genetic changes that could be responsible.

There are also the following risk factors:

• frequent contact with insecticides
• frequent contact with certain herbicides, formaldehyde, benzene, heavy metals and asbestos
• radioactive radiation
• Presence of so-called monoclonal gammopathy of undetermined significance (MGUS)

People diagnosed with MGUS have a one percent chance of developing multiple myeloma later. In contrast to multiple myeloma, the plasma cells in this syndrome also produce more antibodies, but without displacing blood formation, destroying the bone substance or damaging the kidneys. The frequency of MGUS increases with age. In addition, regular occupational contact with pesticides, especially the insecticide dieldrin, the smoking mixture carbon tetrachloride / carbon disulfide and the fungus chlorothalonil, seems to increase the risk of MGUS.

Plasmacytoma diagnosis: this is how multiple myelomas are diagnosed

In addition to inquiring about the patient's medical history and current complaints as well as a thorough physical examination, blood and urine tests play a decisive role in the diagnosis of multiple myeloma.

A plasmacytoma is often discovered by chance as part of a routine examination in which certain laboratory values ​​(creatinine, calcium, albumin, total protein, hemoglobin) were determined. For example, a strong acceleration in the rate of sedimentation, signs of anemia, changes in the blood protein composition and signs of kidney dysfunction are common. If there are abnormalities in the blood values ​​that indicate a lymphatic disease, a targeted search is made for corresponding changes (e.g. determination of antibodies and proteins in the blood and urine using serum protein electrophoresis and quantitative immunoglobulin determination).

In addition to determining laboratory values, X-ray examinations of the long tubular bones, the pelvis, the ribs and the skull as well as a puncture of the bone marrow are carried out. Magnetic resonance imaging (MRI) can show small clusters of tumor cells called focal lesions in the bone and bone marrow. If there is a suspicion that individual organs have been attacked, tissue samples (biopsies) can be taken and examined under the microscope.

In order to control the course of the disease, certain blood and urine values ​​(protein and antibody concentrations) are measured regularly.

How can multiple myeloma be treated?

The therapy of multiple myeloma (plasmacytoma, Kahler's disease) depends on the stage of the disease.

Plasmacytoma stage I or non-symptomatic disease

Patients with stage I multiple myeloma and patients who do not have any symptoms do not initially require treatment. However, the course of the disease is checked regularly in order to notice any progression in good time.

Plasmacytoma stage II, III or symptomatic disease

Treatment is usually initiated in the more advanced stage of multiple myeloma or when symptoms occur. The aim is to achieve a long-term disappearance of the diseased plasma cells or to maintain or improve the quality of life through the treatment.

High-dose chemotherapy and stem cell transplantation

An intensive therapy aimed at the long-term disappearance of the pathologically altered plasma cells (long-term remission) is high-dose chemotherapy with subsequent blood stem cell transplantation. The diseased plasma cells are destroyed by high doses of chemotherapeutic agents. However, since this also damages the blood-forming cells in the bone marrow, the patient then receives blood stem cells back through a transplant, from which new blood is to be formed.

These stem cells come either from the patient himself (taken before therapy, cleaned of diseased cells and then returned – so-called autologous stem cell transplantation) or from a suitable donor (allogeneic stem cell transplantation). Since this is a burdensome and high-risk treatment method, it is primarily the patient's age and general state of health that determine whether high-dose chemotherapy and blood stem cell transplantation are possible or not.

Alternative drug therapy for multiple myeloma

If intensive therapy is not possible due to the patient's age or state of health, therapy with thalidomide, bortezomib or lenalidomide and a combination chemotherapy of melphalan and prednisone (cortisone) can be carried out. This treatment has been shown to increase survival and disease-free time and improve quality of life. It is also used for relapses.

Therapy of bone damage

If there is already bone damage, supportive long-term therapy with bisphosphonates can be carried out. These have a pain-relieving effect, stop further bone destruction and prevent bone fractures. More recent studies have also shown that bisphosphonates (like the active ingredient zoledronic acid) also act directly against tumor cells and therefore contribute to a prolongation of survival times regardless of their effect on the skeletal system. Radiotherapy is also possible for particularly severe bone pain.

Can plasmacytoma be prevented?

There are no specific recommendations for the prevention of plasmacytoma, but tips for certain risk groups.

People who are frequently exposed to insecticides, phenoxy herbicides, formaldehyde, benzene, heavy metals or asbestos, for example, should be aware of their increased risk of multiple myeloma and should inform their doctor if they experience unclear general complaints. The same applies to people whose families have multiple myeloma.

Regular follow-ups are recommended for patients with monoclonal gammopathy of undetermined significance (MGUS) and patients who have been found to have a plasmacytoma but who have no symptoms. If the disease goes into a stage that requires treatment, the necessary therapy can then be initiated in good time.