Myeloma - Cancer is a disease of the blood that is characterized by lesions of B-lymphocytes and the development of these oncological changed plasma cells that synthesize antibodies.Myeloma is also characterized by various manifestations in humans.There multiple myeloma or diffuse myeloma, which is characterized by the development of multiple lesions in the body.There is also a myeloma blood, which is characterized by a decrease in blood viscosity and impaired formation of blood cells.And there are, for example, myeloma bone, which is characterized by an exceptional lesion of bone structures.
Actually, the disease got its name because the malignant myeloma cells infiltrate the bone marrow in a variety of tubular bones and cause them to multiple lesions (diffuse myeloma, myeloma or bone marrow).By the way, the name of the disease, "myeloma" comes from the Greek, where "mielos" means "bone marrow" and ending "ohm" - is the common name for all tumors.Myeloma is literally translated
specific causes that have led to the development of the disease is not present.
believed that the etiological factors of myeloma can be ionizing (radioactive) radiation, oil, asbestos, benzene.Danger prerequisite for myeloma appeared in the human body, it is the age - the average age group for this disease - 65 years.It is found that most often affects men myeloma.
And because of what appear malignant myeloma prototypes?First you need to understand the mechanism of formation of B-lymphocytes, plasma cells, progenitors.
before conversion into plasma cells in the lymphocyte through the following stages.
in the bone marrow pluripotent (the source of all subsequent cell types) stem cell turns into a precursor B lymphocytes (pre-B lymphocyte).Then, pre-B lymphocyte proteins under various stimulator is transformed sequentially, first in B lymphoblasts (immature B-lymphocyte), and then transformed into itself lymphoblast B-lymphocyte.
Next, the formation of plasma cells: B lymphocyte reaches lymph node and settles into the fabric of its follicle, forming a "germinal centers" - secondary follicles reproduction, where they form cells plazmoblasty migrating to the medulla of lymph node, the way of finally passing proliferation and maturing inplasma cells.Synthesis of plasma cells is triggered when the body alien information, namely the foreign antigens.Then macrophage "represents" In this information-lymphocytes and activate the synthesis of plasma cells.You also need to remember that all the daughter cells will remember all the information that was in their parent's B-lymphocytes.
fully formed and healthy plasma cells have one feature not found in other cells: they are able to produce antibodies to a variety of dangerous substances for the body (a sort of "outsiders" or antigens).These antibodies are known as immunoglobulins, and there are 5 types.
Thus, B-lymphocytes by macrophages, memorize a foreign agent attacked the body.He further gives rise to its subsidiaries plasma cells and gives his "memory of a stranger" to them and determine which of the five types of immunoglobulins by plasma cells synthesize need to win and remove the foreign antigen from the body.
In myeloma, B-lymphocytes still can generate new plasma cells which can synthesize immunoglobulins.But there is a problem: immunoglobulins of such cells uniform in structure.This is due to the principle of myeloma tumor cells: all the cells of the tumor cell-born ancestor of one and bear the same information.We can say that the place of normal B lymphocytes occupy the abnormal cells, which begin to synthesize identical plasma cells with the same type of information.This kind of "Attack of the Clones": the plasma cells can not attack infectious and foreign agents, because they do not receive from their progenitor cells no information about the strangers, that is, roughly speaking, the cells are obtained by "stupid."They gather in the bone marrow and the substrate itself is formed of myeloma.And such myeloma lesions may appear in many bones, where there is this type of bone marrow.Typically, myeloma loves to hit the long bones.
And as a result, the body appears:
1. clones of plasma cells that produce the same type of immunoglobulins.They are called monoclonal (literally "from one clone") paraproteins.Develops vulnerability to infections.
2. There is a similar accumulation of these immunoglobulins in the bloodstream, urine and their negative impact on the individual organs.Most often affects the kidneys, increases blood viscosity.
3. Pathological myeloma cells produce osteoklastiruyuschy factor that triggers the destruction of bone structures in the body and the effects of elevated blood level, due to the destruction of bones of calcium.
4. Cells clones collected in the bone marrow and the substrate appears myeloma.Occurrence mielomaticheskih outbreaks in various tubular bones of the body.
in the pathogenesis of which is characterized by myeloma, using two stages of the flow.
first - it is chronic.It myeloma cells have a low capacity for development.It is not depressed the formation of other cells in the bone marrow (myeloma depression).
second stage - acute or terminal.Myeloma tumor cells mutate, causing its clones with increased reproduction.As a consequence, may be suppressed by all the germs of education formed elements, and myeloma metastasized throughout the body.In addition myeloma can develop into other types of cancer (such as lymphosarcoma).
myeloma flow is divided into the following steps (these steps are similar to those of pathogenesis):
1. Asymptomatic period: no changes in the status of patients, yet no clinical signs of disease, but in the urine (usually at randomFor example, routine inspection) can determine the presence of protein (proteinuria).
2. crisis period: There will be expressed all the characteristic clinical symptoms, which starts myeloma.
3. End-stage: there myeloma metastasis to other organs.Oppression of other structures of the bone marrow, the accession of secondary infection, cachexia body.
myeloma also divided into stages of the process and the severity of clinical manifestations: on the level of calcium in the blood, hemoglobin concentration (as verified by the activity of myeloma bone marrow depression), radiographic signs of bone destruction.
hemoglobin level exceeds 100.
calcium indicators are normal.
Radiological findings: no bone destruction, or have a single focus.
low level of paraprotein.
hemoglobin level is 85 to 100. The significance of calcium.
Radiological findings: there is decay and destruction of the bone (osteolysis and osteodestruktsiya).
hemoglobin level low - less than 85 grams in 1 liter.
extremely high calcium content.
Radiological expressed osteodestruktsii multiple processes.
Also, for each stage of the creation of two sub A and B. They are separated by the presence or absence of impaired renal function, namely on the level of serum creatinine.So, Step A is characterized by normal levels of blood serum and step B - increased its level.
Clinical manifestations of myeloma is caused by the following factors: infiltration of bone marrow and bone tumor plasma cells (myeloma bone), the secretion of monoclonal paraprotein and spread of the tumor outside the bone marrow.
The clinic, which is accompanied by myeloma, decided to allocate the following syndromes (symptom complexes):
1. syndrome, bone damage;
2. loss of the hematopoietic system (blood myeloma);
3. kidney damage;
4. syndrome is visceral pathology;
5. syndrome secondary immunodeficiency (secondary infection);
6. hyperviscosity syndrome blood;
7. neurological syndrome;
8. hypercalcemic syndrome (consequences of the massive destruction of the bone).
syndrome bone pathologies
One of the main manifestations of the syndrome is myeloma bone damage (bone myeloma).Its manifestations are explained by bone lesions tumor clones, increased activity of osteoclasts and the development of cell clones factors that stimulate osteoclasts.
Appear pain often suffer from the plasma cells of bone chest.At first, the pain is not constant, decay rapidly.But with an increase in disease activity in pain become unbearable, and their physical activity starts or torso twists.By the way, holding the tip of the index finger tapping on the bones can easily identify their pain.Besides chest affected vertebrae and long bones (long bones).Therefore, the complaint also possible to localize the pain in these areas.
bone lesions often hit flat and short bones (skull, pelvis, sternum, vertebrae).Because myeloma bone will be shown the following X-ray picture: "fishbone" or the spine of the type "bamboo stick" - flattened vertebral bodies.This will lead to a decrease in "settling" the growth of the patient, and the bones on x-ray will be a sort of "moth-eaten", "knocked out punch."These are signs of massive osteoporosis and osteodestruktsii.By the way, in diffuse forms of the disease (diffuse myeloma) is observed only osteoporosis.
Since the normal structure of the bone is broken, it is very often the patients currently receive various fractures: and it does not need to put considerable effort, enough to get just a little bruise.
damaged hematopoietic system
Myeloma Blood develops more at the terminal stages, when the tumor plasma cells inhibit the growth of its other shoots hematopoiesis.
for this syndrome is characterized by the appearance of anemia: it is possible to observe a characteristic "anemic" pale skin.If the process progresses over time, the blood will develop neutropenia and thrombocytopenia.It appears syndrome "pancytopenia" in which the tumor is pushed out of the bone marrow hematopoietic all sprouts.
kidneys are affected due paraprotoinov deposits in the kidneys.Appears Bence Jones proteinuria (identification myeloma Bence-Jones protein in the urine).This all leads to the development of multiple myeloma and renal amyloidosis.In addition, some types of toxic and myeloma paraproteins, laying in the kidneys, can easily cause renal failure.
syndrome of visceral pathologies
This syndrome occurs when the breakout of myeloma in the body.They are easy to damage various visceral (internal) organs.The most "tasty" authority for myeloma getting liver and spleen, and for her.This is easily verified by means of palpation: the liver and spleen are very large in size than their normal value.
In more rare cases of myeloma may infiltrate into the lungs.Then myeloma will be a consequence of pulmonary hemorrhagic effusion.It is also possible secondary damage myeloma skin, resulting in dissemination onkokletok the body.
syndrome secondary immunodeficiency
Since normal immunoglobulins that protect the body from foreign antigens due to the effects of myeloma missing, the body becomes vulnerable to various infections.Often, they have a reason, and death in patients with myeloma: the body have nothing to protect themselves from infection.Due to the pathological effects of bacterial complications of myeloma appear.Heavier flow cystitis and pyelonephritis.Also, the syndrome may progress pancytopenia.Operation of neutrophils is reduced, and hence, is broken and its protection function against infections.
syndrome increased blood viscosity
At the heart of increased blood viscosity is high concentration of myeloma protein in the bloodstream.Consequently, it violated the microcirculation and blood comes barely, or not at all comes to parts of the body.This explains the following symptoms: feeling of "crawling chills" through the body, a variety of visual disturbances, dizziness.These symptoms may lead to the development akrogangreny.
addition, paraproteins myeloma "envelop" platelets and prevent them from performing the function of aggregation.As a consequence, there is a syndrome of increased bleeding: skin hemorrhages appear, may bleed or mucous membranes begin nosebleeds.
Syndrome neurological disorders
Since plasma cells can attack and dura mater, to violate the integrity of the bones of the skull, hitting vertebrae compress the roots, it will be attended by such phenomena: there are paresthesias, "goose bumps" on the body, numbness, may interfere with the functionhands or feet, muscle weakness may appear, can decrease sensitivity to pain.
syndrome increased hypercalcemia
Since in the body there is massive destruction of myeloma bone, then exits from the tissue and calcium accumulation in the blood.Excessive excess of normal levels of calcium leads to vomiting, nausea.It may also be violated consciousness and somnolence.Crystals of calcium deposited in the kidneys and cause interstitial nephrocalcinosis and renal failure.
diagnostic criteria for myeloma
there will be two main criteria: the presence of more than 10 percent of the plasma cells in the bone marrow punctate, its infiltration and plasma electrophoresis detection of monoclonal immunoglobulin.Diagnosis of myeloma can be established only when the combination of these two criteria.
Without a doubt, the main place in the treatment of symptoms that the body has myeloma, chemotherapy is given, and hematologists developed two schemes for the treatment of myeloma:
1. Driving MR.It uses a combination of the following medications: Melphalan / Prednisone + Alkeran.Melphalan and Prednisone accepted from the 1st to the 4th day of chemotherapy, the dose of prednisolone with a 5-day treatment reduced and canceled on the 9th day.Then a break of up to 6 weeks, and the scheme is repeated.This scheme is the "gold standard" treatment for myeloma.
2. The circuit M2.It uses Vincristine Cyclophosphamide + + + Prednisolone Alkeran.This scheme is suitable for patients older than 65 years, or for the treatment of aggressive forms of myeloma.
also now good results in the treatment of myeloma gives bisphosphonates.Particularly well these drugs treat lesions that render the body myeloma bone.The drugs can inhibit the activity of osteoclasts, to stop the destruction of the bone and reduce the spread of the myeloma.Representatives of a number of drugs are Bonefos, Bondronat, Areda.
Treatment of accompanying symptoms of myeloma
great attention to correcting the physiological processes of the body that have undergone as a result of exposure to the myeloma itself (such as bone damage) and indirectly (syndrome of hyperkalemia, which occurs as a consequence of the impact of myeloma in the bone structure).
myeloma treatment of hypercalcemia
Hypercalcemia is treated according to its severity.Mild better hydration of the body to heal apply: up to 3 liters of mineral water a day.At high degrees of calcium administered by intravenous infusion, and combine them with the intravenous administration of Furosemide.This procedure should be done by controlling the parameters of potassium.