Plasmacytoma

All information given here is only of a general nature, tumor therapy always belongs in the hands of an experienced oncologist!

Synonyms

multiple myeloma, Kahler's disease, Kahler's disease, Kahler's disease

definition

A multiple myeloma, which is also synonymous as Plasmacytoma is referred to is a Malignant disease (tumor) of the B lymphocytesthat to the white blood cells belong..

The B-lymphocytes are part of the human immune system and mainly come into the Lymph nodes and in the blood before.

By definition, plasmacytoma is one of the Non-Hodgkin lymphoma with low malignancy (viciousness) and is characterized by the formation of defective immunoglobulins.

Summary

The illness Kahler's disease Like many illnesses, it was named after its discoverer, the Viennese doctor Otto Kahler.
Kahler's disease is also medical Plasmacytoma or multiple myeloma called.

This is a malignant disease affecting the white blood cells in the Bone marrowwhich can also affect organs outside of the bone marrow as the disease progresses. In this case, one speaks of an extramedullary multiple myeloma.

Plasma cells belong to the group of white blood cells, too Leukocytes called. There are different types of white blood cells in our bodies, all of which are busy fighting off intruders (e.g. viruses and bacteria).
Plasma cells form what are known as immunoglobulins, substances that bacteria and viruses can mark in order to make them recognizable to the body's own phagocytes.
At the multiple myeloma usually only one type of plasma cell proliferates (hence the expression: monoclonal = starting from a single trunk). This means that at one point in time one plasma cell degenerates and multiplies. So it forms many identical images of its own cell.
Since it is important to be able to react to the many different attackers on the human body, we need many different plasma cells. However, since monoclonal plasma cells all produce the same immunoglobulins (defense indicators, partly defective or incomplete), a specific defense is no longer possible.
The defective immunoglobulins (repellants) are also called Paraproteins or M proteins designated.
Some of these are incomplete protein chains that can be detected in the blood with the help of electrophoresis. These protein chains will too Bence Jones egg white or Bence Jones proteins called. Tumors that produce these proteins will also Light chains - myeloma called.
Particularly degenerate forms of plasmacytoma can no longer produce defensive proteins. It is so-called non-secreting myeloma.

The plasmacytoma does not set any actual Metastases. Tumor cell clusters can affect internal organs. If the multiple myeloma grows strongly in the bone marrow, bone dissolution (lysis) can occur. This weakens the bone and can lead to so-called pathological fractures. Pathological fractures are fractures caused by tumor growth.

frequency

Overall, plasmacytoma is a rare disease. The incidence, i.e. the rate of new cases per year, is around 3 per 100,000 inhabitants. Men get sick slightly more often than women.

Men are more frequently affected than women; an occurrence before the age of 60 is unusual, but possible.

root cause

As already described above, plasmacytoma / multiple myeloma belongs to the group of low-grade non-Hodgkin lymphomas. Low malignant means: slightly malignant. However, this does not refer to the forecast, but to the growth behavior.

The cause is considered to be the malignant degeneration of a B lymphocyte. This primordial cell multiplies in an uncontrolled manner and creates identical duplicates of its own cell. This genetic relationship can be demonstrated in the blood and is used for diagnosis through the detection of monoclonal antibodies.

Even after intensive research, the development of the plasmacytoma has not yet been clarified in all stages.
Many blood cancers are related to viral diseases, immune system disorders or chemotherapy. For multiple myeloma, no evidence of any involvement of these factors in the development has been found so far.
However, one inheritable component is known.

Symptoms

There are no specific symptoms that are definitely indicative of multiple myeloma. To the general symptoms counting:

• A frequently occurring feature is an impairment of the general condition general feeling of illness, Fatigue, weight loss and a slightly elevated temperature
• Step in the majority of patients Bone pain on. They are often the first sign that patients lead to the doctor. This bone pain occurs particularly frequently in the area of ​​the spine (Back pain) on. They are caused by the uncontrolled growth of tumor cells in the bone and the slow skeletal destruction induced it can lead to one Vertebral fracture come. This destruction is also considered on the X-ray Bone damage to recognize. The one pictured above X-ray image of the skull shows sharply demarcated bone dissolutions (Osteolysis), which are typical of Kahler's disease.
• Because of a too high calcium levels or the thickening of the blood can cause confusion, and nausea Vomit come. However, this only occurs more frequently in the final stages of the disease.
• Due to the disturbed immune system, there is an increased susceptibility to infection.

If protein chains are produced, these can kidney clog. Consequence can be Kidney failure be.
Due to the overproduction of monoclonal antibodies, these can be deposited throughout the body. So-called amyloid can be found in many organs. This Amyloid clogs the kidney ducts and prevents the filtration function.

metastasis

In most cases the plasmacytoma spreads diffusely throughout the bone marrow and is therefore more or less detectable everywhere. In areas of high activity, so-called osteolytic foci (bone damage) are visible on the X-ray.
Spread to other organs is rare. In most cases, lymph nodes are involved.

Complications

Listed below are common problems and complications that can occur with plasmacytomas / multiple myelomas:

1. Pathological fractures:
   Pathological fractures are spontaneous fractures that are caused by the bone damage from the plasma cells.
   A sudden onset of pain can be a sign of a vertebral fracture. The osteolyses are the result of the release of messenger substances by the degenerated plasma cells. These stimulate the cells (osteoclasts) that break down the bone substance (see above).
2. Too much calcium in the blood:
   The attacks on the bones can also lead to an increase in the level of calcium in the blood (hypercalcemia) and the associated symptoms such as thirst, tiredness, nausea and vomiting.
   The increased calcium can be deposited in the kidneys during excretion and thus lead to kidney damage, as can the above-mentioned proteins (monoclonal immunoglobulins, Bence Jones proteins). Sooner or later, these are deposited in the kidneys and damage them.
3. The stem cells of the red blood cells are displaced by the tumor growth of the plasma cells in the bone marrow. This leads to anemia (anemia). For the same reason, a blood platelet deficit can also develop, which increases the risk of bleeding. The immune system can lead to a disruption of the immune system through impaired production of immunoglobulins. Therefore, the risk of infection is increased, from which the patient recovers less quickly.

forecast

As a result of medical research and improved therapeutic options, the prognosis for Kahler's disease has improved considerably in recent years.
Nevertheless, the plasmacytoma is nowadays with a few exceptional cases not curable.
The months and years of complaint and symptom-free times are becoming increasingly longer. Unfortunately, in almost all cases there is a renewed activity of the disease and a so-called relapse (relapse).

A fundamentally new form of therapy that would make plasmacytoma curable is currently not in sight. Nevertheless, with a continuous improvement of the therapy options, an extension of the survival time will become possible.

In some cases, a plasmacytoma can turn into a acute leukemia pass over. This worsens the general prognosis.