Multiple myeloma, also known as plasmacytoma or Kahler’s disease, is a malignant disease of the lymphatic system. This leads to an excessive accumulation of pathologically modified plasma cells in the bone marrow. Find out more about the symptoms, treatment options and life expectancy.
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Quick overview: Frequently asked questions and answers
How does multiple myeloma manifest itself? The first symptoms are very non-specific. For example, you may experience constant fatigue, night sweats, and weight loss. For most people affected, multiple myeloma is noticeable through bone pain, especially in the back.
Is multiple myeloma curable? To date, multiple myeloma is generally not curable. Thanks to new treatment options, the disease can be easily contained and quality of life maintained.
What is the prognosis for multiple myeloma? The life expectancy of those affected has improved significantly in recent years. On average, 58 percent of women and 56 percent of men are still alive 5 years after diagnosis.
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What is multiple myeloma?
Multiple myeloma is characterized by many tumor foci in the bone marrow. If only one focus of disease is present, it is a solitary (isolated) plasmacytoma. Multiple myeloma is one of the so-called non-Hodgkin lymphomas, a group of cancers that originate from cells in the lymphatic system.
Multiple myeloma arises from the degeneration of a single plasma cell in the bone marrow. Plasma cells are B lymphocytes and are white blood cells. They play a role in the specific immune defense.
The result is an excessive proliferation of plasma cells in the bone marrow. This leads to a disruption in blood formation and a weakening of the immune system with increased susceptibility to infections. The myeloma cells also influence bone metabolism and there is a risk of osteoporosis and bone fractures.
Good to know:
Previously, the terms multiple myeloma and plasmacytoma were used interchangeably. Today, experts only speak of a plasmacytoma, although in fact there is only a solitary tumor made up of plasma cells. The name Kahler’s disease, after the Viennese doctor Kahler who first described the disease, is no longer in use today.
Frequency of multiple myeloma
In Germany, around six to eight out of 100,000 inhabitants develop multiple myeloma every year. This makes it one of the 20 most common types of cancer in Germany. The frequency increases with age; those affected are usually over 70 years old when diagnosed. Men are affected slightly more often than women.
Symptoms: How does multiple myeloma manifest itself?
Multiple myeloma usually begins with no symptoms and progresses gradually over a period of a few weeks to months or even years. Initially, uncharacteristic general complaints occur.
Possible signs are:
- Bone pain (especially in the spine area)
- Loss of performance and weakness
- fatigue
- increased susceptibility to infections
- Loss of appetite, weight loss
- feeling thirsty,
- Night sweats
- light fever
These symptoms can also indicate other illnesses. Nevertheless, such complaints should always be clarified by a doctor.
Course of the disease in multiple myeloma
As the disease progresses, further symptoms may arise. Possible complications include:
Dissolution of the bone substance: Painful bone fractures can occur as a result of bone damage (skeletal lesions).
Disturbance of kidney function: Possible symptoms include very foamy urine, frequent urge to urinate with particularly large amounts of urine or the cessation of urine production (uremia).
Blood formation disorder: If the myeloma cells spread in the bone marrow and displace blood-forming cells, this can lead to anemia. This manifests itself, among other things, in shortness of breath during exertion (for example when climbing stairs) and pale skin.
peripheral neuropathies: Main symptoms are Sensitivity disorders and abnormal sensations – especially in the legs.
increased calcium levels in the blood: A light one Hypercalcemia usually causes no symptoms and is not dangerous. However, if the calcium concentration is excessive, symptoms such as nausea, vomiting, muscle weakness, drowsiness and even coma can occur. This is an emergency that must be treated immediately.
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Causes of multiple myeloma
The causes of plasmacytoma have not yet been conclusively researched. However, there are some known factors that increase the risk of developing the disease.
Risk factors for multiple myeloma:
- African descent
- frequent contact with certain chemicals (such as insecticides, formaldehyde, benzene, heavy metals and asbestos)
- radioactive radiation
- familial clustering
- older age
MGUS: precursor to multiple myeloma
People diagnosed with monoclonal gammopathy of undetermined significance (MGUS) have a one percent per year chance of later developing multiple myeloma. The precancerous condition is usually discovered as an incidental finding.
MGUS is not a disease. In this syndrome, the plasma cells also increasingly form monoclonals Blood proteins (immunoglobulins), although this is an asymptomatic condition. However, monoclonal gammopathy of unclear significance can develop into multiple myeloma.
The frequency of MGUS increases with older age. In addition, regular occupational contact with pesticides or other chemicals appears to increase the risk of MGUS.
Plasmacytoma diagnosis: This is how multiple myelomas are diagnosed
In addition to inquiring about your medical history and current complaints as well as a thorough physical examination, blood and urine tests play a crucial role in the diagnosis of multiple myeloma. A plasmacytoma is often discovered accidentally as part of a routine examination.
If multiple myeloma is suspected, the following tests will be carried out:
Blood test (important parameters include beta-2 microglobulin, creatinine, LDH, C-reactive protein and light chains in serum)
Analysis of the 24-hour urine collection to determine protein secretion
X-ray examination of the long bones, pelvis, ribs and skull
Taking a tissue sample from the bone marrow (biopsy)
Whole-body computed tomography (whole-body CT)
Magnetic resonance imaging (whole body MRI)
In order to monitor the progression of the disease, certain blood and urine values (protein and antibody concentrations) are measured regularly.
Treatment of multiple myeloma
So far, a cure is usually not possible. The aim is therefore to
- to slow down the progression of the disease as best as possible,
- to avoid complications and
- to maintain the quality of life of those affected.
The treatment of multiple myeloma depends, among other things, on the age, the state of health of those affected and the stage of the disease. Treatment is usually initiated when symptoms arise or there is a threat of immediate organ damage.
High-dose chemotherapy and stem cell transplantation
An intensive therapy that aims at the long-term disappearance of the pathologically modified plasma cells (long-term remission) is high-dose chemotherapy followed by blood stem cell transplantation. The pathological plasma cells are destroyed by high doses of chemotherapy drugs.
However, since this also damages the blood-forming cells in the bone marrow, patients are then given back blood stem cells through a transplant, from which new blood is supposed to form. 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 suitable donors (allogeneic stem cell transplantation).
Overall, it is a stressful and risky therapeutic procedure. Therefore, age and general health primarily determine whether high-dose chemotherapy and blood stem cell transplantation are possible or not.
Alternative therapies with medication
If intensive therapy is not possible due to age or health, therapy with medication can be carried out.
Substances that can be used are:
- Thalidomide
- Bortezomib
- Lenalidomide
- Cyclophosphamide
- Daratumumab
- Dexamethasone
They are usually used in combination with chemotherapy. This treatment has been shown to increase survival and disease-free time and improve quality of life. It is also used in the event of disease relapses.
Additional therapy for bone damage
If bones have already been damaged, long-term supportive therapy with bisphosphonates can be carried out. These work
- pain relieving,
- stop further bone destruction and
- prevent bone fractures.
Recent studies have also shown that bisphosphonates (such as the active ingredient zoledronic acid) also act directly against tumor cells and therefore contribute to an extension of survival times, regardless of their effect on the skeletal system. If bone pain is particularly severe, radiation therapy is also possible.
New forms of therapy for multiple myeloma
Numerous new, promising therapies for bone marrow cancer are currently being developed. These include, for example:
CAR T cell therapy: Those affected receive genetically modified immune cells that destroy previously undetected cancer cells in the body. Cell therapy has so far only been approved for patients who have had a relapse or have not responded to previous therapies.
- Antibody therapy: Patients receive antibodies (often in combination with other medications) that target protein structures on the surface of myeloma cells.
What is the prognosis for multiple myeloma?
The prognosis for multiple myeloma has improved significantly in recent years. With high-dose chemotherapy and stem cell transplantation, the disease can often be well controlled and the disease-free periods (remission) can be significantly extended.
However, permanent healing is only possible in very rare cases. The 5-year relative survival rate is 58 percent in women and 56 percent in men.
Patients with certain gene changes in myeloma cells have an increased risk of a more aggressive course of the disease. In order to better assess this, cytogenetic examinations (looking at chromosomes) are also carried out as part of the diagnosis.
However, new forms of therapy raise the hope that treatment success can continue to improve or even cures can be made possible.
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