Plasmocytoma (multiple myeloma, Kahler’s disease) is a rare, low-malignant bone marrow tumor for which there are as yet no therapeutic measures that condition a complete cure. In this regard, the rate of disease increases beyond the age of 50, and men are more commonly affected by plasmacytoma than women.
What is a plasmocytoma?
Plasmocytoma (also multiple myeloma, Kahler’s disease) is the name given to a rare, low-malignant (less malignant) tumor disease that originates primarily from a degenerate plasma cell of the bone marrow (known as medullary plasmocytoma) and, in rare cases, may also manifest in extramedullary cell tissue (pharynx, lung, stomach). In plasmocytoma, multiple tumors (myelomas) originating from the bone marrow are often present and proliferate, thereby causing symptoms characteristic of plasmocytomas, such as weight loss, lassitude, local pain, anemia due to displacement of hematopoietic tissue by progressive spread of the tumor, spontaneous fractures due to skeletal destruction, and multiple bone defects (especially in the skull).
Causes
The causes for the development and manifestation of plasmacytoma are as yet unclear. It is considered certain that plasma cell proliferation originates from a cell clone (degenerated plasma cell) that malignantly proliferates in the bone marrow. The resulting tumor cells of the plasmocytoma cause an excessive production of pathological immunoglobulins without antibody function (so-called monoclonal antibodies or paraproteins), which are useless for the body’s own defenses. As the disease progresses, the growing tumor tissue displaces the hematopoietic tissue and causes the anemia typical of plasmacytoma. The triggers for this degeneration process are unknown. Due to the fact that plasmocytomas originate from a cell clone and occur frequently in certain families, a genetically determined predisposition (disposition) is assumed. In addition, ionizing radiation (electromagnetic or radioactive radiation) and some chemical substances (pesticides) are considered risk factors for plasmacytoma.
Symptoms, complaints, and signs
The leading symptom of plasmocytoma is bone pain, which is caused by an attack of the vertebral bodies or other bones by the cancer cells. The pain usually increases with movement and improves with rest. The cancerous involvement of the bones further causes bone fractures, which often go unnoticed, especially in the spine. It is not uncommon for bone complaints to be misinterpreted as osteoporosis or even rheumatism. Anemia (anemia) also develops in early stages. [[Facial pallor|pallor], fatigue, a limited ability to perform and concentrate, as well as an increased tendency to infection are the result. Since these symptoms tend to develop insidiously, they often go unnoticed at first. The patients’ immune system is weakened by a lack of white blood cells. They suffer more from infections and are prone to complications. As the malignant disease gradually makes the bones more porous, more calcium accumulates in the blood. The increased calcium level can impair the function of the kidneys. 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, punctate hemorrhages (petechiae) occur.
Diagnosis and course
Because a plasmocytoma causes no symptoms or only minor discomfort at the beginning of its development, it is diagnosed by chance in many cases. Affected individuals, however, stand out because of characteristic symptoms such as anemia or frequent infectious diseases. The diagnosis of a plasmocytoma is made on the basis of a bone marrow biopsy and histological evidence of plasma cell proliferation (more than 10 percent plasma cells in the bone marrow smear). In the course of an immunoelectrophoresis (analysis of plasma proteins), the typical paraproteinuria (increased concentration of paraproteins in the urine) can be detected.An X-ray examination detects potential bone defects, while a blood and/or urine analysis can be used for laboratory diagnosis to detect signs of anemia, renal insufficiency, and accelerated BKS (erythrocyte sedimentation rate). The course and prognosis of a plasmacytoma are determined primarily by the stage of development of the tumor. The earlier the tumor is diagnosed and treated, the higher the life expectancy of the affected person, although a complete cure is not yet possible and plasmocytoma has a recurrent course.
Complications
In plasmocytoma, affected individuals suffer from a tumor in the bones. This can lead to death if it is not treated. 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 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 also suddenly considerably reduced. Patients also suffer from fever and night sweats. Furthermore, pain occurs in the bones, with the back being particularly affected. Without treatment, bone loss occurs due to the spread of the tumor. Patients also suffer from a weakened immune system due to the plasmacytoma and thus more frequent infections or inflammations. Furthermore, renal insufficiency occurs and eventually the affected person dies. The treatment of plasmocytoma is carried out with the help of surgical interventions and stem cell transplantation. However, it cannot be universally predicted whether this will result in a completely positive course of the disease.
When should you see a doctor?
Symptoms such as headache, pallor, physical weakness, and fatigue indicate plasmacytoma. A visit to the doctor is indicated if the typical signs of illness do not resolve on their own within two to three days. If the symptoms become more severe, a doctor must be consulted. Affected individuals are best advised to speak to their family doctor or an internist. If there is damage to the bone substance, the orthopedist must also be consulted. A nutritionist can work out a diet with the patient to reduce kidney damage. It is suspected that people who have occupational contact with pesticides are particularly susceptible to the disease. A family history of the disease is also a warning sign that should be investigated in conjunction with the above symptoms. Elderly people, pregnant women, and people with a history of cardiovascular disease should always see a doctor if the above symptoms occur. If kidney failure, severe infection, or other medical emergency occurs as a result of plasmacytoma, emergency medical services must be called.
Treatment and therapy
The therapy of a plasmocytoma depends on the stage of development (spread, size, localization, accompanying symptoms) of the tumor. Often, a plasmocytoma is monitored at the beginning of its development (stage I) by close follow-up examinations and, for the time being, chemotherapy, which entails burdensome side effects, is not given. In advanced stages (stages II and III), chemotherapy and radiotherapy are usually used for plasmacytoma, with the two forms of therapy being combined due to the presence of multiple tumor foci. If the person affected by a plasmocytoma is in a corresponding general condition, high-dose chemotherapeutic agents with subsequent stem cell transplantation may also be therapeutically indicated. Chemotherapy destroys not only the tumor cells but also the stem cells (blood-forming cells). As a result of stem cell transplantation, these can rebuild and recover. If the plasmocytoma cells have certain antigens on their surface, antibody therapy can be used. In this case, the administered antibodies attack the antigens of the tumor cells and destroy the cells of the plasmocytoma. In some cases, in addition to chemotherapy and following stem cell transplantation, thalidomide is used to inhibit tumor growth and bisphosphonates are used to stabilize bone metabolism.Surgery may be required for plasmacytoma to prevent paralysis due to nerve damage.
Prevention
Because the causes of plasmacytoma have not yet been determined, no preventive measures exist. In principle, contact with known risk factors such as ionizing radiation or pesticides and other carcinogenic substances should be avoided.
Follow-up
In the case of plasmacytoma, affected individuals usually have only a few and usually limited measures of follow-up care available to them. Affected individuals should therefore seek medical attention at the first symptoms of this disease to prevent complications or other medical conditions from developing as the disease progresses. An early diagnosis with subsequent treatment usually always 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, patients are dependent on taking various medications, whereby attention must always be paid to correct dosage and also regular intake. If there are any uncertainties or side effects, it is always advisable to consult a doctor first. Many of those affected are dependent on the help and support of the family due to the plasmocytoma. Building up conversations can also prevent the development of depression and other psychological complaints. In some cases, the plasmacytoma can thereby reduce the life expectancy of the affected person.
Here’s what you can do yourself
It is imperative that this serious disease be treated by a doctor, even though a complete cure is not yet possible. However, the disease and its course can be stopped or alleviated with medical measures, so it is essential to follow their instructions. Those affected are also advised to check their working environment. Are toxins or pesticides being processed or used in the workplace? If so, patients would need to consider changing their workplace. Patients will still benefit from living as healthy a lifestyle as possible so as not to put additional stress on the body. Extended rest periods, little stress, regular 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, affected patients should drink plenty of water to better eliminate toxins. The diagnosis of plasmocytoma (multiple myeloma) is very stressful for those affected, especially if they are undergoing chemotherapy in advanced stages of the disease and/or are waiting for a stem cell donation. Psychotherapeutic supportive treatment would be advisable here. Relaxation techniques such as Reiki, yoga, mediations and breathing exercises or progressive muscle relaxation according to Jacobson can be supportive. Tapping acupressure (EFT) is also a good self-help measure, especially for patients prone to anxiety attacks.
