Multiple myeloma


Multiple myeloma

Description Multiple Myeloma

Multiple myeloma is a cancer caused by the proliferation(growth in the number) of plasma cells and their accumulation in the bone marrow.

Plasma cells are the final stages in the development of B cells(an important type of white blood cells that are responsible for producing antibodies). Tumors of lymphocytes, is called. Lymphomas.

Accurate pathological sign of multiple myeloma is a malignant non-Hodgkin’s lymphoma, which has its own characteristic structure, which is determined under a microscope. Analysis of the structure of the tumor under a microscope for the specialist – physician-pathologist. A pathologist under a microscope can identify different types of cancer (plazmocytární, plazmoblastový and mixed).

The tumor affects 3-4 people per 100,000 population per year.Both sexes are affected about equally. Plasmocytoma represents 1% of all malignant tumors and 10% of all malignant hematological tumors (tumors of the blood and bone marrow).

 

Causes and risk factors for multiple myeloma

Generally, it is not known to cause disease, but 90% of patients exhibits genetic change – the mutation of certain genes.

The greatest risk factor is age: multiple myeloma is a disease of elderly people, with the majority of patients aged 61-70 years. 3% of patients, however, ill under the age of 40 years.

Other factors are human exposure to radiation and chemicals. We can not exclude a genetic predisposition (susceptibility to disease at the genetic level).

 

Prevention of Multiple Myeloma

Specific prevention does not exist, it is recommended adherence to healthy lifestyle: appropriate diet, plenty of exercise, mental health, meaningful work, steer clear of alcohol, nicotine and drugs.

If possible, avoid exposure to chemicals and radiation (see Risk Factors).

 

Signs and symptoms of multiple myeloma

Multiple myeloma mainly affects the bone marrow in which are formed bearings which destroy bone material. These bearings are referred to as osteolytic.

The principle of the formation of deposits is described below. Osteolytic lesions are stored mainly in the ribs, sternum, in the vertebrae, in the pan and in the bones of the skull, but in principle may be anywhere. Locations locating bearings are consistent with the occurrence of so-called. Red bone marrow of an adult (a place where an adult human consists of blood cells).

The osteolytic lesions destroys adjacent bone structure, thereby weakens, which can causepathological fracture. Pathologic fracture is a fracture that is produced without the use of pressure or tension on the bone or the forces are minimal. On formation of normal fracture affects only congestion tension and pressure on which a given bone is not adapted. Pathologic fracture can occur without injury.

From the presence of osteolytic lesions arise other symptoms (signs) of the disease: a protracted back pain (if damaged vertebrae – called. Vertebrogenic algie, which are lingering back pain around the spine).

Resorption (absorption) in myeloma bone tissue leads to the release of large amounts ofcalcium (calcium) in the blood. Bone resorption occurs on the preponderance of extinction bone over its origins. For the formation of new bone cells called special responsibility. Osteoblasts. On the contrary extinction bone care multinucleated osteoclasts destroying bone to enzymatic digestion in microscopic cavities (cavities are technically called Howshipovy lacunae).

It should be noted that even in a healthy body leads to constant degradation and the formation of new bone tissue activities aforementioned two types of cell lines. The entire process is called bone remodeling. In myeloma leads to increased bone resorption. Osteoclasts are stimulated with a substance called OAF (osteoclast-activating factor).

From bone resorbed is released calcium (calcium). There is a condition called. Hypercalcemia, thus increasing the amount of calcium in the blood above the reference limit. This state can be easily diagnosed in routine laboratory tests.

Other symptoms resulting from the above fact that the tumor cells are plasma cells (with an abnormal genetic information). These tumor plasma cells differ from normal (physiological) cells, first mutations in the DNA, which causes some genes that normally control the activities of other genes are mutated and thus non-functional or functional too.

The process can be likened to a military unit: the whole body of troops (a group of genes coding for certain substances) is subordinate to a single commander (in our case a control gene that their products checked the work of subordinates genes. Thus, the mutation of a single control gene cause figuratively abnormal function of many genes subordinates.

It must be added that this situation is only outlined a gross oversimplification issues regarding genes and their expression. All mutations are reflected in the behavior of the plasma cells, which becomes a malignant cell. Such a cell is divided almost unlimited, requires growth factors such as normal cells. Radically changing their metabolism. It is dampened by a process called apoptosis.

Apoptosis is next necrosis type of cell death. While necrosis can be translated as cell death kill (the exposure cells pollutants that have greater intensity than the possibility of reparation mechanisms of cell-ie. Cell pollutant kills) and apoptosis can be translated as cell suicide. Tumor cell then loses the ability to suicide, so they may continually divide. Furthermore it destroys normal cells and later whole tissue.

Tumor cells produce antibodies (immunoglobulins) or fragments thereof in large quantities. These antibodies are often broken or it is only part of the chains of the antibody molecule. The antibody consists of two heavy chains (H chain, from the English. Heavy) and two light chains (L- chain, again from Eng. Light). These chains are joined by disulfide bridges (the chemical bonds which contain 2 sulfur atoms). These antibodies are receiving blood to the kidneys, where gradually clogthe filtration apparatus of the kidney. If the clogging of the filter, there is a failure, renal insufficiency talking about.

In the case of myeloma then such insufficiency kidney called myeloma kidney. Kidney insufficiency then not able to remove waste and excess substances from the body, which body very damaging.

Antibodies that arise in myeloma are formed by a single clone of pathological cells (clones are identical cells that arise so that initially there is a malignant transformation of a single cell which then divides and gives rise to cells subsidiary that divide further, but all tumor cells are the same – they are clones of a single parent tumor cells). Therefore, these antibodies are called monoclonal antibodies.

These antibodies secondly clog the filter in the kidney, but partially penetrate into urine. The presence of protein in urine is known as proteinuria. Typically there is a transfer of the urine only light chains (L-chains of immunoglobulins), because these molecules are so small that they pass partly into moči.Tato protein, consisting of 2 light chains are referred to as Bence Jones proteins.

For comprehensive information, it must be said that the efferent tubules in the kidneys, which are connected to the filter membrane kidneys are filled with Bence-Jones protein, producing castings such kanálků- we call them war.

As already mentioned, the antibodies are defective, and therefore patients there is dysfunction of the immune system- a so called. Immunodeficiency. Patients then suffer more infections which are more severe than in otherwise healthy people and tend to return repeatedly.

Another symptom of the disease is anemia or anemia. The causes of anemia are now two: the first is that the bone marrow produces red blood cells (erythrocytes), and myeloma bone marrow damages, therefore, produces little erythrocytes. The second cause of the damage to the kidneys (see above). Some kidney cells because they produce a substance called erythropoietin, which acts as a growth stimulatory factor for erythrocytes. Erythropoietin (also known as EPO-doping agentwhich functions to increase the number of red blood cells, which then carry more oxygen to tissues, leading to increased power) is formed in the body of 90% in kidney cells, the remaining 10% consists of Kupffer cells in the liver.

Multiple myeloma may also manifest fatigue and weakness. The causes of fatigue and weakness are anemia (body has less oxygen), then it is hypercalcemia (electric currents affecting the membranes of neurons, muscle cells, etc.) And substantially damaging the complex organism tumor.

The specific phenomenon in myeloma is the fact that his clinical picture does not include symptoms which are present in most other haematological malignancies: it is primarily elevated temperature, below the patient very sweaty during the night, quickly lose weight, has an enlarged liver and spleen – called. Hepatosplenomegaly and he has called. lymphadenopathy,which is an enlargement of the lymph nodes.

The above description applies to the so-called. Classic multiple myeloma. The disease has several less frequently occurring variants: a) the non-secretory myeloma: it is the classic myeloma, but already the name suggests, it does not produce antibodies.
b) smoldering myeloma (SMM also from Eng. smoldering myeloma) is a disease that is characterized by benign (benign) process, which is stable with regard to antibody production and the presence of pathological clone of plasma cells. We find no organ damage or osteolytic lesions.
c) indolent myeloma: the SMM is similar, but there are rare osteolytic lesions
d) plasmocellular leukemia is when we find more than 20% plasma cells in the blood, there is a massive infiltration of the bone marrow. Plasmacellular leukemia rarely occur as a primary disease, but it is a kind of terminal (final) stage of classical myelomas.

Summary of all typical symptoms of multiple myeloma: fatigue, weakness, anemia, hypercalcemia, pain, kidney damage, proteinuria and damaged immunity. If these symptoms are present in patients in the critical age group (see above) can be used several tests (see below), as the diagnosis of multiple myeloma confirm or disprove. 15% of patients is revealed only upon routine examination of the patient, which is performed for other reasons, or in preventive inspections: this is especially blood count, biochemistry, and sedimentation.

 

Treatment of Multiple Myeloma

At the outset it must be said that multiple myeloma is a currently incurable disease. To complete recovery occurs very rarely. Typically, physicians may induce withdrawal treatment nemoci- called.Remission, which, however, after a certain time and terminated disease strikes again – relapse.For classical myelomas occur several remissions and relapsům- is thus protracted illness.

Each treatment has a specific target or group of targets as well as a tactic to achieve the objectives.Treatment goals are: induction of remission (resting period of illness), eliminate symptoms of the disease, increase the standard of living of the patient.

Tactics therapy consists in two directions: a) anti-tumor therapy: it tries to destroy the greatest number of tumor cells. In the context of antitumor therapy distinguish radiation (i.e. radiation of the tumor), chemotherapy (treatment with anticancer drugs), and the last one is the possibility of high doses of antitumor agents and subsequent bone marrow transplantation.

The last mentioned treatment modality is intended administration of large amounts of drugs which destroy almost all tumor cells. Treatment, however, is destroyed and bone marrow, therefore, there must be a transplant, or the patient did not survive this treatment. Anticancer drugs known ascytostatics.

Different types of treatments may be combined and dosed at certain time intervals. During treatment, it is necessary to realize that most of the procedures are also toxic to normal cells of the human body. Therefore, there is a secondary symptoms of treatment: liquidation hematopoiesis leads to anemia, infections, mucosal damage, pain, loss of appetite, loss of hair etc.

b) supportive therapy: focuses on treating the symptoms of multiple myeloma. It does not therefore aim to liquidate tumor cells. It is the administration of bisphosphonates, which are drugs that prevent decalcification of the bone and also have the effect of damping in the form of bone pain.

Furthermore, it is indicated rehabilitation exercises or for positively affecting the state of bone and muscle.

Very important it is to administer pain-relieving medications (analgesics), because there is no rational reason to keep a patient in pain, often very cruel. The doctor through analgesic therapy significantly increases the standard of living of the patient, which is also the general trend of medicine as a whole.

 

Prognosis (perspective) human multiple myeloma

It is very unfavorable, because the disease is still back, and ultimately leads to death of the patient. The average survival of patients diagnosed with multiple myeloma is less than three years.10% of patients survive more than 10 years, which is considered a great success. If the bone marrow infiltration by tumor cells at a rate greater than 50%, it’s a very bad sign and a life expectancy of a man falling rapidly.

 

How can I help myself

Multiple myeloma is a very serious disease that the patient himself very little influence. DBAN can recommend strict guidelines for medical staff, sleep, intake of enough fluids, which helps protect the kidneys.

 

Complications of Multiple Myeloma

Result from impaired hematopoiesis (bone marrow produces blood cells: red blood cells, some white blood cells and platelets) leads to the development of anemia, low white blood cells caused by faulty antibodies along with weakened immunity, therefore, are developing infections. Few platelets may occur bleeding.

Further, it may cause bone fractures (see above).

Other names: plasmacytoma, plazmocytární myeloma, Kahler’s disease, morbus Kahler

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