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 after the age of 50 and men are more frequently affected by a plasmacytoma than women.
What is a plasmacytoma?
A plasmacytoma (also multiple myeloma, Kahler’s disease) is a rare, low-grade (less malignant) tumor disease, which primarily originates from a degenerated plasma cell of the bone marrow (so-called medullary plasmacytoma) and in rare cases also develops in the extramedullary cell tissue (pharynx, lungs, stomach) can manifest. See sportingology for Meaning of Mesenteric Lymphadenitis in English.
In the case of a plasmacytoma, there are often multiple tumors (myeloma) originating from the bone marrow, which multiply and thus the symptoms characteristic of plasmacytoma, such as weight loss, exhaustion, local pain, anemia as a result of the displacement of the hematopoietic tissue due to the progressive spread of the tumor, spontaneous fractures due to skeletal destruction as well as multiple bone defects (particularly in 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 starts 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), which are useless for the body’s own defense. As the development progresses, the growing tumor tissue displaces the hematopoietic tissue and causes the anemia typical of a plasmacytoma.
The triggers for this degeneration process are unknown. Due to the fact that plasmacytomas start from a cell clone and occur frequently in certain families, a genetic predisposition (predisposition) is assumed. In addition, ionizing radiation (electromagnetic or radioactive rays) and some chemical substances (pesticides) are considered risk factors for a plasmacytoma.
Symptoms, Ailments & Signs
The main symptom of plasmacytoma is bone pain, which is caused by cancer cells invading the vertebral bodies or other bones. The pain usually increases with movement and improves with rest. Bone fractures, which often go unnoticed, especially in the area of the spine, also occur due to the cancer infestation of the bones.
Bone problems are often misinterpreted as osteoporosis or rheumatism . Anemia also develops in the early stages. The result is [[facial pallor|pallor], tiredness, a limited ability to perform and concentrate, and an increased tendency to infections. Since these symptoms tend to develop insidiously, they often go unnoticed at first.
The patient’s immune system is weakened by a lack of white blood cells. They suffer more from infections and are prone to complicated disease progression. Since the bones gradually become more porous as a result of the malignant disease, more calcium accumulates in the blood.
The function of the kidneys can be impaired by the increased calcium level. The patients feel tired, listless 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 bleeding (petechiae) occur.
Diagnosis & History
Since a plasmacytoma causes no symptoms or only minor symptoms at the beginning of its development, it is diagnosed accidentally in many cases. However, those affected are conspicuous by the characteristic symptoms such as anemia or frequent infectious diseases.
The diagnosis of a plasmacytoma is based on a bone marrow biopsy and 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 can detect potential bone defects, while a blood and/or urine analysis can be used to diagnose signs of anemia, renal insufficiency and accelerated BKS (blood sedimentation rate).
In the case of a plasmacytoma, the course and prognosis are primarily determined by the developmental stage of the tumour. The earlier the tumor is diagnosed and treated, the higher the life expectancy of the person affected, although a complete cure is not yet possible and a plasmacytoma has a recurrent course.
Complications
In the case of a plasmacytoma, those affected suffer from a tumor in the bones. This can lead to death if left untreated. The further course and the 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 complaints and symptoms of this disease are not particularly characteristic, so that this tumor is often diagnosed late.
Patients suffer primarily from severe fatigue and exhaustion. The resilience of those affected is suddenly significantly reduced. The patients also suffer from fever and night sweats. Pain in the bones also occurs, with the back being particularly affected. Without treatment, bone loss occurs as the tumor spreads.
Patients also suffer from a weakened immune system due to the plasmacytoma and thus more often from infections or inflammations. Furthermore, it comes to renal failure and finally to the death of the person concerned. Plasmacytoma is treated with the help of surgical interventions and stem cell transplantation. However, it cannot be universally predicted whether the course of the disease will be completely positive.
When should you go to the doctor?
Symptoms such as headaches, paleness, physical weakness and tiredness indicate a plasmacytoma. A doctor’s visit is indicated if the typical symptoms of the disease do not go away on their own within two to three days. If the symptoms get worse, a doctor must be consulted. Affected people are best advised to speak to their family doctor or an internist. In the case of damage to the bone substance, the orthopedist must also be consulted. A nutritionist can work with the patient to develop a diet that will reduce kidney damage.
It is believed that people who come into contact with pesticides at work are particularly susceptible to the disease. Familial accumulation is also a warning sign, which must be clarified in connection with the symptoms mentioned. Elderly people, pregnant women and people with a previous cardiovascular disease 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 services must be called.
Treatment & Therapy
The therapy of a plasmacytoma depends on the stage of development (spreading, size, location, accompanying symptoms) of the tumor. A plasmacytoma is often observed at the beginning of its development (stage I) by means of close-meshed follow-up examinations and chemotherapy, which entails stressful side effects, is dispensed with for the time being.
In advanced stages (stages II and III), chemotherapeutic and radiotherapeutic 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 has a corresponding general condition, high-dose chemotherapeutic agents with subsequent stem cell transplantation can 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. Antibody therapy can be used if the cells of the plasmacytoma have certain antigens on their surface. The administered antibodies attack the antigens of the tumor cells and destroy the cells of the plasmacytoma.
In some cases, in addition to chemotherapy and following a stem cell transplant, thalidomide is used to inhibit tumor growth and bisphosphonates are used to stabilize bone metabolism. Surgery may be necessary to avoid paralysis due to nerve damage in the case of a plasmacytoma.
Prevention
Since the causes of a plasmacytoma have not yet been 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 usually have only a few and usually only limited follow-up measures available. Those affected should therefore consult a doctor as soon as the first symptoms of this disease appear, so that there are no complications or other symptoms as the disease progresses. Early diagnosis and subsequent treatment usually have a very positive effect on the further course of the disease.
The treatment itself depends heavily on the severity of the plasmacytoma, so that no general course can be given. In most cases, however, the patients are dependent on taking various medications, whereby it is always important to ensure the correct dosage and regular intake. If there are any uncertainties or side effects, it is always advisable to consult a doctor first.
Because of the plasmacytoma, many of those affected are dependent on the help and support of their families. Constructive conversations can also prevent the development of depression and other mental health problems. In some cases, plasmacytoma can reduce the life expectancy of those affected.
You can do that yourself
This serious disease must be treated by a doctor, even if a complete cure is not yet possible. However, the disease and its course can be stopped or alleviated with medical measures, which is why their instructions must be followed. Those affected are also advised to check their working environment. Are toxins or pesticides processed or used in the workplace? If this is the case, patients would have to consider changing jobs.
Patients continue to benefit from a lifestyle that is as healthy as possible so as not to put additional strain on the body. Extended rest periods, little stress, regulated routines, 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 a lot of water in order to be able to eliminate toxins better.
The diagnosis of plasmacytoma (multiple myeloma) is very stressful for those affected, especially if they are being treated with chemotherapy in advanced stages of the disease and/or are waiting for a stem cell donation. Here, a psychotherapeutic accompanying treatment would be advisable.
Relaxation techniques such as Reiki, yoga, mediation and breathing exercises or progressive muscle relaxation according to Jacobson can have a supportive effect. Tapping acupressure (EFT) is also a good self-help measure, especially for patients who are prone to anxiety attacks.
