Multiple myeloma and plasmacytoma: what is behind it?

Multiple myeloma is also known as Kahler’s disease,  Kahler’s disease or (colloquially) plasmacytoma and is a form of bone marrow cancer. Those affected usually only notice later in the course of the disease that they are suffering from multiple myeloma. Bone pain or anaemia are the most common symptoms. A blood test can diagnose Kahler’s disease. There has been significant progress with new therapy concepts in recent years, contributing to a longer life expectancy. Read here how multiple myeloma develops, the typical symptoms, and the treatment options.

What is multiple myeloma?

Multiple myeloma is a cancer that affects the plasma cells of the bone marrow. These plasma cells belong to the white blood cells and are part of the body’s immune system.

Multiple myeloma belongs to the group of lymphomas. Physicians summarize several malignant tumours that arise from lymphatic system cells as lymphomas. The lymphatic system includes all lymphatic channels and various lymphatic organs, such as the lymph nodes, the spleen or the thymus gland.

Colloquially, one also speaks of bone or bone marrow cancer since the lymphatic system cells develop in the bone marrow and mature there.

 

What causes multiple myeloma?

To better understand the cause of multiple myeloma, we need to look closer at our bone marrow. There are immature stem cells of the lymphatic system, which can transform into so-called B or T cells through maturation. These two cells are of elementary importance for our immune system because they fight pathogens. For example, as part of an immune reaction, B cells convert into plasma cells that produce specific antibodies so that invaders can be attacked in a more targeted manner.

If an error occurs during cell division and a degenerated plasma cell develops,  this can result in multiple myeloma. Further “clones” of this degenerate cell form. These plasma cells produce ineffective antibodies and displace other healthy cells from the bone marrow. Various tumours, i.e. malignant growths, develop in the bone marrow from the non-functional plasma cells. This has consequences for a wide range of functions in the body – which is why multiple myeloma can cause a wide range of symptoms.

How does a plasmacytoma develop?

The terms plasmacytoma and multiple myeloma are sometimes used interchangeably. Strictly speaking, these are two different diseases. While a single, clearly defined tumour forms in a plasmacytoma, which can also occur outside of the bone marrow (extramedullary), in multiple myeloma, the degenerated plasma cells affect many parts of the skeleton, resulting in several small tumours in the bone marrow.

Both forms, multiple myeloma and plasmacytoma, belong to the group of non-Hodgekin lymphomas, including around 30 different lymphoma diseases.

 

Causes: What are myeloma triggers?

Multiple myeloma is a relatively rare disease. It is assumed that there are three to four new cases of illness per 100,000 inhabitants in Germany (incidence). Those affected are, on average, 70 years old at the time of diagnosis.

The exact reason why the plasma cells change and Kahler’s disease develops is still unknown. Possible risk factors are radioactive radiation or certain immune diseases (congenital immune defects, some autoimmune diseases, HIV ).

Is multiple myeloma hereditary?

The guidelines on multiple myeloma describe that familial accumulation and, thus, heritability are rare. However, it cannot be completely ruled out since first-degree relatives have an increased risk of developing multiple myeloma.

It has also been found that the incidence (new cases per year) is twice as high in black US populations as compared to Caucasian populations.

What are the symptoms of multiple myeloma?

Multiple myeloma can remain asymptomatic for a long time. If symptoms develop during the disease, they are very diverse. Some of them are:

• bone pain
• Anaemia: This is usually noticeable through paleness, exhaustion, tiredness, shortness of breath during exertion and headaches
• Susceptibility to infections because the number of functional white blood cells decreases
• foaming urine
• Kidney dysfunction can manifest as an increased accumulation of water in the tissues ( oedema ).
• B symptoms: Doctors use this term to summarize three symptoms:
  • Fever
  • night sweats
  • weight loss

Early detection of multiple myeloma based on symptoms is difficult. The most common complaints are pain in the spine,  which is often initially mistaken for back pain. Multiple myeloma initially spreads to the bones. This leads to a thinning of the bone substance, which promotes fractures.

 

How does the doctor make a diagnosis?

Since multiple myeloma can sometimes cause very unspecific symptoms, the diagnosis is usually made using blood values. In addition, however, the examination of the urine and other examinations can also play a role in the diagnosis of multiple myeloma.

blood test

The blood sedimentation rate (ESR) often provides the first clue. This involves observing how quickly the red blood cells sink in a particular tube within an hour. Elevated ESR values ​​indicate inflammatory processes in the body.

Furthermore, an increased calcium value and a lack of red and white blood cells and platelets can be noticed. The last circumstance is also known as pancytopenia.

Another blood test is immunofixation electrophoresis. In this test, protein components in the blood are sorted according to their size. There are five different factions. In the case of multiple myeloma, serum electrophoresis shows that the last fraction, the so-called gamma fraction, which contains the antibodies, is abnormally high. This is known in laboratory medicine as monoclonal gammopathy.

urine test

A so-called Bence Jones proteinuria is found in the urine of many of those affected. The degenerated plasma cells produce ineffective antibodies, also excreted in the urine. Antibodies consist of proteins that cause the urine to foam if the concentration is too high.

 

Further investigations

In addition to the blood and urine tests, other tests are carried out. These include apparatus-based procedures such as a low-dose CT (computed tomography), which requires only a little X-ray radiation and no contrast medium. This examination discovers areas in the skeletal system where the bone material has already severely dissolved (osteolysis). The CT also helps to assess the stage of the disease better. An MRI (magnetic resonance or magnetic resonance imaging) is also performed.

To finally make the diagnosis, however, the doctors have to carry out a biopsy or bone marrow puncture  (i.e. removal of bone marrow from the pelvic bone with a kind of punch) so that they can examine the material obtained under the microscope.

Stage critical to treatment

Once multiple myeloma has been diagnosed, it is divided into stages. Many diagnostic criteria are summarized for this purpose, for example, whether it is a plasmacytoma with a tumour, multiple myeloma with different tumour foci, or whether symptoms are already occurring. The choice of therapy depends on this.

Therapy of multiple myeloma

Therapy for multiple myeloma is always highly individual. Depending on the condition of the patient and the course of the disease, the following treatment measures (sometimes in combination) can be considered:

• close monitoring without therapy in asymptomatic patients
• High-dose chemotherapy in advanced or rapidly progressing cases
• Autologous bone marrow donation: Bone marrow is removed from the patient before chemotherapy and then transplanted.
• Maintenance therapy: After the high-dose chemotherapy, so-called maintenance therapy with Revlimid® can be carried out. This maintains the therapeutic success achieved with chemotherapy over a more extended period. Revlimid®, which contains the active ingredient lenalidomide, inhibits the development of tumour cells.
• Radiation therapy to reduce bone pain
• Administration of bone-building drugs such as bisphosphonates
• blood transfusions

The treatment options listed are just a selection and do not apply to every patient. The doctor treating you should discuss which therapy is suitable with the person concerned in each case.

 

What diet is advisable for multiple myeloma/plasmacytoma?

Many cancers result in significant weight loss. Chemotherapy is also a strain on the body, which is why a wholesome, balanced diet is generally recommended for cancer patients. Lots of fruit and vegetables, whole grain products, and an adequate protein intake are part of such a diet.

Dietary supplements and other supplements should be clarified with the doctor treating you.

How else can you support the treatment?

Other additional options for improving the quality of life are, for example, appropriate pain therapy and an adequate vaccination status to protect against infections.

But also, self-help groups, in which those affected can exchange their experiences with the disease, are a valuable addition to the therapy measures.

Can you cure bone cancer?

Treatment aims to stop the progression of the disease to such an extent that the symptoms disappear as far as possible. Despite improved therapy, a cure is usually not possible. Instead, physicians are striving for remission. This means that the disease is no longer detectable, and those affected are as symptom-free as possible. However, this is not to be equated with a cure.

The survival rate and course of the disease depend on many factors. To receive optimal therapy, it is advisable to visit specialized centres.

 

Prognosis: how long can you live with multiple myeloma/plasmacytoma?

Life expectancy in multiple myeloma is very variable. In the final stage, it can only be a few months, while other sufferers live with the disease for more than ten years. The prognosis is, therefore, not easy to make and very individual.

An essential factor is existing organ damage. In the case of multiple myeloma, one does not necessarily speak of metastases, but the disease can also affect other organs in addition to the skeleton. For example, in the context of amyloidosis, protein fragments (so-called light chains) that originate from the degenerated plasma cells are deposited in the body and can lead to lead to heart failure.

In the final phase of the disease, the body – especially the immune system – is severely weakened. The cause of death can, therefore, be an infection or acute kidney failure. You can never predict how you will die from multiple myeloma.

How to deal better with the topic of dying from cancer is the speciality of oncological psychologists. You can help those affected by this difficult situation.

The life expectancy of those affected by multiple myeloma or plasmacytoma has improved significantly in recent years. New drugs and new therapy concepts have been successfully put into practice. Many other new approaches are currently in the testing phase. Even if multiple myeloma cannot be cured, there is reason to hope it can be treated even better.