Rustitzky-Kahler's disease or myeloma is an oncological disease of the circulatory system. A characteristic feature of the ailment is that because of a malignant tumor in the blood, the number of plasmocytes (cells that produce immunoglobulins) increases, which begin to produce a large amount of pathological immunoglobulin (paraprotein).
Multiple myeloma - what is it in simple words?
Multiple myeloma is one of the forms of myeloma. Plasmocyte-striking tumor in this disease occurs in the bone marrow. Statistically, myeloma of the bones of the spine, skull, pelvis, ribs, thorax, and, more rarely, tubular bones of the body, is more common. Malignant formations (plasmacytomas) with multiple myeloma capture several bones and reach a size of 10-12 cm in diameter.
Plasmocytes are a constituent part of the body's immune system. They produce specific antibodies that protect against a specific disease (which immunoglobulin should be produced by "prompting" special memory cells). Plasma cells infected with a tumor (plasmomyeloma cells) uncontrolled produce incorrect (damaged) immunoglobulins that can not protect the body, but accumulate in some organs and disrupt their work. In addition, the plasmacytoma causes:
- decrease in the number of erythrocytes, platelets and leukocytes;
- increased immunodeficiency and increased vulnerability to various infections;
- hematopoiesis and blood viscosity increase;
- violation of mineral and protein metabolism;
- the appearance of infiltrates in other organs, especially often in the kidneys;
- pathological changes in bone tissue in the tumor region - the bone is thinned and destroyed, and when the tumor sprouts through it, it penetrates into soft tissues.
Causes of myeloma
The illness of Rustitskiy-Kahler has been studied by doctors, but there is no consensus on the reasons for its occurrence in medical circles. It was found that in the body of a sick person, lymphatic viruses of T or B type are often present, and since plasma cells form from B-lymphocytes, any violation of this process leads to failure and the onset of formation of pathoplasmocytes.
In addition to the viral version, there is evidence that myeloma can also be triggered by radiation exposure. Doctors studied people affected in Hiroshima and Nagasaki, in the explosion zone at the Chernobyl nuclear power plant. It was found that among those who received a high dose of radiation, the percentage of cases of myeloma and other diseases affecting the blood and lymphatic system is high.
Among the negative factors that increase the risk of contracting myeloma, doctors call:
- Smoking - the longer the experience of the smoker and the more the number of cigarettes smoked, the higher the risk;
- immunodeficiency;
- impact on the body of toxic substances;
- genetic predisposition.
Myeloma - symptoms
Myeloma mainly occurs in old age, affecting both women and men. The disease Rustitskogo-Kahler - the symptoms and clinical picture, observed in patients:
- defeat hemopoietic and bone systems;
- violation of metabolic processes;
- pathological changes in the urinary system.
Multiple myeloma symptoms:
- the earliest signs of myeloma are pain in the bones (in the spine, chest, skull bones), spontaneous fractures, deformation of bones and the presence of tumor formations;
- frequent pneumonia and other diseases, provoked by the fall of immunity and restriction of respiratory movements caused by changes in the bones of the chest;
- dystrophic changes in the muscles of the heart, heart failure;
- increased spleen and liver;
- myeloma nephropathy - a disorder in the kidneys with a characteristic increase in proteins in the urine, which translates into renal insufficiency;
- normochromic anemia - decrease in the number of erythrocytes and hemoglobin level;
- hypercalcemia - an increased amount of calcium in the plasma and in the urine, this condition is very dangerous, its symptoms - vomiting, nausea, drowsiness, vestibular apparatus disruptions, mental pathologies;
- decrease in the level of normal immunoglobulin;
- hemopoiesis disorders - bleeding of mucous membranes, bruising, spasms of arteries phalanges, hemorrhagic diathesis;
- paresthesia ("creepy"), headaches, drowsiness, turning into a stupor, convulsions, dizziness, deafness, dyspnea;
- at the last stages - weight loss, fever, serious anemia.
Forms of myeloma
According to the clinical-anatomical classification, myeloma is of the following forms:
- solitary myeloma - with one tumor in the bone or lymph node;
- multiple (generalized) myeloma - with the formation of several tumor lesions.
In addition, multiple myeloma can be:
- diffuse - in this case pathologically the formations have no boundaries, but permeate the whole structure of the bone marrow;
- multiple-focal - the plasmacytoma of the bone develops in limited areas, and in addition, tumors can occur in the lymph nodes, spleen.
- diffuse focal - combining the symptoms of diffuse and multiple.
Myeloma disease - stages
Doctors subdivide three stages of multiple myeloma, the second stage is transitional, when the indices are higher than in the first, but lower than in the third (the heaviest):
- The first stage is characterized by a hemoglobin lowered to 100 g / l, normal calcium level, low concentration of paraproteins and Bens-Jones protein, one tumor focus of 0.6 kg / m², no osteoporosis, bone deformation.
- The third stage is characterized by a lowered to 85 g / l and lower hemoglobin, a calcium concentration in the blood above 12 mg per 100 ml, multiple tumors, a high concentration of paraproteins and the Bens-Jones protein, a total tumor size of 1.2 kg / m² or more, signs of osteoporosis.
Complications of myeloma
For multiple myeloma, complications associated with the destructive activity of the tumor are characteristic:
- severe pain and fracture of bones (fractures);
- renal failure with the need for hemodialysis ;
- persistent infectious diseases;
- anemia in a serious stage, requiring transfusions.
Myeloma - diagnosis
With the diagnosis of myeloma, differential diagnosis is difficult, especially in cases where there are no obvious tumor foci. The patient is examined by a hematologist who suspects a diagnosis of myeloma, who first conducts a survey and finds out whether there are such signs as bone pain, bleeding, frequent infectious diseases. Further, additional studies are made to clarify the diagnosis, its shape and extent:
- general analysis of blood and urine;
- chest and skeletal x-ray;
- CT scan;
- biochemical blood test ;
- coagulogram ;
- a study of the amount of paraproteins in the blood and urine;
- bone marrow biopsy;
- study on the method of Mancini on the definition of immunoglobulins.
Myeloma - a blood test
If a diagnosis of myeloma is suspected, the doctor prescribes a general and biochemical blood test. The following indicators are typical for the disease:
- hemoglobin - less than 100 g / l;
- erythrocytes - less than 3.7 t / l (women), less than 4.0 t / l (male);
- platelets - less than 180 g / l;
- leukocytes - less than 4,0 g / l;
- ESR - more than 60 mm per hour;
- protein - 90 g / l and above;
- albumin - 35 g / l and below;
- urea - 6.4 mmol / L and higher;
- calcium - 2.65 mmol / l and higher.
Myeloma - X-ray
The most important stage of the study with myeloma is X-ray. The diagnosis of multiple myeloma diagnosis using radiography can fully confirm or leave in doubt. Tumors in the x-ray are clearly visible, and in addition - the doctor is able to assess the extent of damage and deformation of bone tissue. Diffusive lesions on the X-ray reveal more difficult, so the doctor may need additional methods.
Myeloma illness - treatment
Currently, for the treatment of myeloma, an integrated approach is used, with the primary use of drugs in various combinations. Surgical treatment is required to fix the vertebrae due to their destruction. Multiple myeloma - drug treatment includes:
- Targeted therapy , stimulating the synthesis of proteins, leading the fight with paraproteins;
- chemotherapy, inhibiting the growth of cancer cells and killing them;
- Immune therapy aimed at stimulating one's own immunity;
- corticosteroid therapy, which enhances basic treatment;
- treatment with bisphosphonates that strengthen bone tissue;
- anesthetic therapy aimed at reducing pain syndrome.
Myeloma - clinical recommendations
Unfortunately, it is impossible to completely recover from myeloma, the therapy is aimed at prolonging life. To do this, you must follow certain rules. Diagnosis of myeloma - recommendations of doctors:
- Carefully observe the treatment prescribed by the doctor.
- Strengthen immunity not only with medicines, but also with walking, water procedures, sunbathing (using sunscreen and during minimal solar activity - in the morning and in the evening).
- To protect from infection - observe personal hygiene rules, avoid crowded places, wash hands before taking medication, before eating.
- Do not walk barefoot, because of the defeat of the peripheral nerves it is easy to get hurt and not notice it.
- Monitor the level of sugar in foods, as some drugs contribute to the development of diabetes.
- Maintain a positive attitude, because positive emotions are of critical importance for the course of the disease.
Chemotherapy for multiple myeloma
Chemotherapy for myeloma can be done with one or more drugs. This method of treatment allows to achieve complete remission in about 40% of cases, partial - in 50%, however, relapses of the disease occur very often, as the disease affects many organs and tissues. Plasmacytoma - treatment with chemotherapy:
- At the first stage of treatment, the chemotherapy prescribed by the doctor in the form of tablets or injections is taken according to the scheme.
- In the second stage, if the chemotherapy is effective, the bone marrow stem cells are transplanted - take a puncture , extract the stem cells and attach them back.
- Between courses of chemotherapy, courses of treatment with interferon-alpha drugs are carried out - to maximize remission.
Multiple myeloma - prognosis
Unfortunately, with the diagnosis of myeloma, the prognosis is disappointing - doctors are only able to prolong the periods of remission. Often patients with myeloma die from pneumonia, fatal bleeding caused by violations of blood clotting, fractures, kidney failure, thromboembolism. A good prognostic factor is young age and the first stage of the disease, the worst prognosis is in people older than 65 with concomitant diseases of the kidneys and other organs, multiple tumors.
Multiple myeloma - life expectancy:
- 1-2 years - without treatment;
- up to 5 years - the average life expectancy of myeloma for people undergoing therapy;
- up to 10 years - life expectancy with good response to chemotherapy and disease in an easy stage;
- more than 10 years can only live patients with a single tumor focus, successfully removed by doctors.