Plasmacytoma (multiple myeloma)

A Plasmacytoma (multiple myeloma, Kahler's disease) is a rare, low-grade bone marrow tumor for which there are currently no therapeutic measures that require complete healing. The incidence rate increases beyond the age of 50 and men are more often affected by a plasmacytoma than women.

What is a plasmacytoma?

A plasmacytoma is diagnosed on the basis of a bone marrow biopsy and the histological evidence of plasma cell proliferation (more than 10 percent plasma cells in the bone marrow smear).
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As Plasmacytoma (also multiple myeloma, Kahler's disease) is a rare, low-grade (less malignant) tumor disease that primarily originates from a degenerate plasma cell of the bone marrow (so-called medullary plasmacytoma) and, in rare cases, can also manifest itself in the extramedullary cell tissue (throat, lungs, stomach).

In a plasmacytoma there are often multiple tumors (myelomas) originating from the bone marrow, which multiply and thus the symptoms characteristic of plasmacytomas such as weight loss, exhaustion, local pain, anemia due to the displacement of the blood-forming tissue by the progressive spread of the tumor, spontaneous fractures due to skeletal destruction as well as causing multiple bone defects (especially on the skull).

causes

The causes of the development and manifestation of a Plasmacytoma have not yet been clarified. It is considered certain that the plasma cell multiplication originates from a cell clone (degenerate plasma cell), which multiplies malignantly in the bone marrow.

The resulting tumor cells of the plasmacytoma cause an excessive production of pathological immunoglobulins that are useless for the body's own defense and have no antibody function (so-called monoclonal antibodies or paraproteins) that are useless for the body's own defense. As development progresses, the growing tumor tissue displaces the blood-forming tissue and causes the anemia (anemia) typical of plasmacytoma.

The triggers for this degeneration process are unknown. Due to the fact that plasmacytomas originate from a cell clone and occur more frequently in certain families, a genetic predisposition (disposition) is assumed. In addition, ionizing radiation (electromagnetic or radioactive radiation) and some chemical substances (pesticides) are risk factors for a plasmacytoma.

Symptoms, ailments & signs

The main symptom of plasmacytoma is bone pain that is caused by the cancer cells attacking the vertebral bodies or other bones. The pain usually increases when you move and improves when you rest. The cancerous attack on the bones also leads to bone fractures, which often go unnoticed, especially in the area of ​​the spine.

It is not uncommon for the bone problems to be misinterpreted as osteoporosis or rheumatism. Anemia (anemia) also develops in the early stages. [[Pallor paleness], tiredness, restricted performance and concentration as well as an increased tendency towards infection are the result. Since these complaints tend to develop slowly, they too often go unnoticed at first.

A patient's immune system is weakened by a lack of white blood cells. They suffer more and more from infections and tend to develop complex disease courses. Since the bones gradually become more porous due to the malignant disease, more calcium accumulates in the blood.

The kidneys can be impaired in their function due to the increased calcium level. The patients feel tired, limp and listless. Some of those affected also complain of nausea and vomiting. In rarer cases, the cancer also affects the formation of blood platelets (thrombocytes). If this is the case, punctiform hemorrhages (petechiae) occur.

Diagnosis & course

There a Plasmacytoma causes no symptoms or only minor discomfort at the beginning of its development, in many cases it is diagnosed by chance. However, those affected are noticeable by the characteristic symptoms such as anemia or frequent infectious diseases.

A plasmacytoma is diagnosed on the basis of a bone marrow biopsy and the histological evidence of plasma cell proliferation (more than 10 percent plasma cells in the bone marrow smear). The typical paraproteinuria (increased concentration of paraproteins in the urine) can be detected as part of an immunoelectrophoresis (analysis of plasma proteins).

An X-ray examination is used to detect potential bone defects, while a blood and / or urinalysis can be used to diagnose the signs of anemia, renal insufficiency and accelerated BKS (blood sedimentation rate).

The course and prognosis of a plasmacytoma are primarily determined by the stage of development of the tumor. The earlier the tumor is diagnosed and treated, the higher the life expectancy of the person affected, although a complete cure has not yet been possible and a plasmacytoma has a recurrent course.

Complications

With a plasmacytoma, people have a tumor in their bones. This can lead to death if left untreated. The further course and complications of this disease depend very much on the spread of the tumor in the patient's body, so that a general prediction is usually not possible.The symptoms and symptoms of this disease are not particularly characteristic, so that this tumor is often diagnosed late.

The patients mainly suffer from severe fatigue and exhaustion. The resilience of those affected is suddenly significantly reduced. The patients also suffer from fever and night sweats. Furthermore, pain in the bones occurs, with the back being particularly affected. Without treatment, bone loss occurs due to the spread of the tumor.

The patients also suffer from a weakened immune system due to the plasmacytoma and thus more often from infections or inflammations. Furthermore, there is renal insufficiency and ultimately death of the person concerned. The treatment of the plasmacytoma takes place with the help of surgical interventions and a stem cell transplant. However, it cannot be universally predicted whether this will lead to a completely positive course of the disease.

When should you go to the doctor?

Symptoms such as headache, paleness, physical weakness, and fatigue suggest a plasmacytoma. A doctor's visit is indicated if the typical symptoms do not subside on their own within two to three days. A doctor should be consulted if the symptoms worsen. Affected people are best to talk to their family doctor or an internist. In the event of damage to the bone substance, the orthopedist must also be consulted. A nutritionist can work out a diet together with the patient and thus reduce kidney damage.

It is believed that people who have work-related contact with pesticides are particularly susceptible to the disease. Familiar accumulation is also a warning sign, which must be clarified in connection with the symptoms mentioned. Elderly people, pregnant women and people with a pre-existing cardiovascular system should always see a doctor if the symptoms mentioned occur. If kidney failure, severe infection, or any other medical emergency occurs as a result of a plasmacytoma, emergency medical services should be called.

Treatment & Therapy

The therapy of a Plasmacytoma depends on the stage of development (spread, size, location, accompanying symptoms) of the tumor. A plasmacytoma is often observed at the beginning of its development (stage I) by means of close-knit follow-up examinations and chemotherapy, which has uncomfortable side effects, is avoided for the time being.

In advanced stages (stage II and III), chemotherapy and radiotherapy measures are usually used for a plasmacytoma, with the two forms of therapy being used in combination due to the presence of multiple tumor foci. If the person affected by a plasmacytoma shows a corresponding general condition, high-dose chemotherapeutic agents with subsequent stem cell transplantation may also be therapeutically indicated. In addition to the tumor cells, the chemotherapy also destroys the stem cells (blood-forming cells).

As a result of a stem cell transplant, these can rebuild and recover. If the cells of the plasmacytoma have certain antigens on their surface, antibody therapy can be used. Here, the administered antibodies attack the antigens of the tumor cells and destroy the cells of the plasmacytoma.

In some cases, thalidomide to inhibit tumor growth and bisphosphonates to stabilize bone metabolism are used in addition to chemotherapy and following stem cell transplantation. Surgery for a plasmacytoma may be required to avoid paralysis due to nerve damage.

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prevention

As the causes of a Plasmacytoma could not yet be clarified, there are no preventive measures. In principle, contact with known risk factors such as ionizing radiation or pesticides and other carcinogenic substances should be avoided.

Aftercare

In the case of a plasmacytoma, those affected generally have only a few and usually only limited follow-up measures available. Those affected should therefore consult a doctor at the first symptoms of this disease, so that there are no complications or other complaints in the further course. An early diagnosis with subsequent treatment usually has a very positive effect on the further course of the disease.

The treatment itself depends strongly on the severity of the plasmacytoma, so that no general course can be given. In most cases, however, the patients are dependent on the intake of various medications, always paying attention to the correct dosage and regular intake. If anything is unclear or if there are side effects, it is always advisable to consult a doctor first.

Many of those affected need help and support from their families because of the plasmacytoma. Building conversations can also prevent depression and other psychological complaints from developing. In some cases, the plasmacytoma can reduce the life expectancy of the person affected.

You can do that yourself

This serious illness must be treated by a doctor, even if a complete cure is not yet possible. The disease and its course can, however, be stopped or alleviated with medical measures, which is why their instructions must be followed. Those affected are still advised to check their work environment. Are toxins or pesticides processed or used in the workplace? If so, patients would have to consider changing jobs.

The patients continue to benefit from a lifestyle that is as healthy as possible in order not to put additional strain on the body. Extended rest periods, little stress, regular processes, as much exercise as possible and a selected diet with fresh, vitamin-rich food, little sugar and fat are recommended. Nicotine and alcohol, on the other hand, are taboo. Instead, the affected patients should drink plenty of water so that they can excrete toxins better.

The diagnosis of plasmacytoma (multiple myeloma) is very stressful for those affected, especially if they are treated with chemotherapy in advanced stages of the disease and / or are waiting for a stem cell donation. An accompanying psychotherapeutic treatment would be advisable here.

Relaxation techniques such as Reiki, yoga, mediation and breathing exercises or Jacobson's progressive muscle relaxation can have a supportive effect. Tapping acupressure (EFT) is also a good self-help measure, especially for patients who are prone to anxiety attacks.