Plasmocytoma (multiple myeloma)

Description

Plasmocytoma is a specific type of blood cancer in which plasma cells of the blood malignantly grow out of control. It belongs to the group of lymphomas, more specifically B-cell non Hodgkin lymphomas. Synonymous names are multiple myeloma or Kahler's disease.

Plasma cells are a subspecies of white blood cells that are responsible for immune defense and are formed in the bone marrow.

They arise from altered plasma cells and produce excessive amounts of proteins (antibodies) that are non-functional (paraproteins). This activity weakens the immune system and makes sufferers more susceptible to infection.

Generally, only 7 out of 100,000 people contract this disease each year, and it affects men more often than women.

Plasma cell out of control

Healthy plasma cells are derivatives of white blood cells. They are produced in the bone marrow and make antibodies that are necessary for the immune system and immune responses.

In a plasmacytoma, a plasma cell reproduces out of control. Gradually, healthy, blood-forming cells in the bone marrow are displaced by the malignant plasmacytoma cells.

The exact cause of this malignant development of plasma cells is not yet fully understood. Changes in chromosomes (components of the DNA), predominantly in chromosomes 13 and 14, are frequently found in cancer cells.

It is also suspected that external influences such as radiation (ionising) and pesticides favour the development of a playmocytoma.

Plasmocytoma patients often develop symptoms only after the disease has been present for a long time. The most common symptom is back pain, as bone substance is broken down and vertebral bodies become brittle.

Since this leading symptom can have many different causes, it often takes a long time before a clear diagnosis is found.

Changes visible in the blood count:

• Anemia (anemia): too few red blood cells - listlessness, shortness of breath under stress, headaches, irritability;
• Leukopenia: lack of white blood cells - increased susceptibility to serious and prolonged infections;
• Thrombocytopenia: too low a platelet count (blood platelets) - increased tendency to bleed, tendency to hematomas (bruises);
• High blood calcium level: due to degradation of bone substance - nausea, vomiting, confusion, rarely vertebral fractures and paralysis;

Further, kidney function is reduced as (para-) proteins clog the renal tubules. Often less fluid can be excreted, the body weight increases and oedemas develop. Kidney failure cannot be ruled out.

Plasmocytoma is divided into stages 1-3. Here, the more advanced the disease, the higher the stage. A number of examinations are necessary for the allocation to the correct stage:

Blood count

A blood test provides the first indications. The focus is on protein content, calcium and kidney values. Paraproteins (monoclonal gammopathy) can also be detected in multiple myeloma.

The degree of deviation from normal values is related to the extent of the disease. Thus, direct conclusions can be drawn from examination results (rising or falling values) about the development of the disease.

Bone marrow aspiration

The plasma cell content of the bone marrow is increased in plasmocytoma from a normal 5% to up to >10%. A more precise examination of the degenerated cells is essential for the choice of therapy and a prognosis that is as realistic as possible.

X-ray

Bone loss in particular (osteolytic foci or osteoporosis) is visible on an X-ray.

Although plasmocytoma can be treated, it cannot be completely cured. The aim of the therapy is to contain the spread of the degenerated plasma cells.

In stage 1, there are often no symptoms yet. Patients are merely observed, as there is no proven benefit from early treatment. If the plasmacytoma progresses, chemotherapy is an option.

For stages 2 and 3, the current standard treatment is high-dose chemotherapy followed by bone marrow transplantation. This is an autologous transplant that uses healthy stem cells from the patient's own body.

If a stem cell transplant is impossible, chemotherapy is combined with the administration of cortisone. Both can be done by taking tablets.

The extremely painful bone changes require supportive therapy. X-ray radiation and medication (bisphosphonates) alleviate pain. Preventive measures against infections are also important. Vaccinations, e.g. against influenza, pneumococci or haemophilus influenzae, are suitable for this purpose.

Relapses (recurrences) occur very often in multiple myeloma, even if the patient has responded very well to the therapy. Follow-up therapies become necessary:

• Chemotherapy: renewed chemotherapy is only useful for patients who have been completely free of plasmacytoma for at least 6 months after successful therapy (complete remission).
• Bortezomib: This is a so-called proteasome inhibitor. It interferes with the metabolism of cancer cells and is approved for the treatment of relapse.
• Lenalidomide, thalidomide: Immunomodulating drugs that prevent the formation of blood vessels in the bone marrow and also influence the metabolism of the plasmacytoma cells.

The type of multiple myeloma, age, general health of the patient and even the division rate of the cancer cells have a significant impact on the prognosis. Life expectancy varies greatly from a few months to several years.

The disease usually becomes chronic after a period of 2-5 years. Death often occurs due to a lack of blood cells. Patients over 60 years of age often die from other causes such as heart attacks or strokes.