There are many diseases that are rare and are found in exceptional cases. One of these pathologies is the Waldenstrom macroglobulinemia.
What is Waldenstrom Macroglobulinemia?
This is a fairly rare bone marrow tumor, formed from the lymphocytic and plasmocyte cells of , for which a typical increase in the size of the spleen and liver.
In addition, the pathology is accompanied by frequent nasal bleeding, the presence of abnormal protein in the blood and anemia.
This is a hereditary disease of the blood, so-called.hematological syndrome, characterized by the presence in the blood of macroglobulin - a protein substance, which is formed due to the activity of B-lymphocytes.
As a result of excessive production of proteins, the blood becomes excessively viscous, which leads to problems of thrombogenesis and bleeding. The velocity of the vascular blood flow decreases, the blood deposits grow, the blood-swallowing ability is inhibited. As a result, thrombus-forming processes are violated, and a tendency to bleeding appears.
In the normal state B-lymphocyte cells participate in the formation of M-globulin, but if the functions of the medullary substance are for some reason violated( usually due to tumors), then active development of macroglobulin occurs.
Causes of the disease
Pathogenetic bases of the disease are caused by the reproduction of malignant cells that promote the production of immunoglobulin elements.
These cells spread to the spleen, liver and bone marrow. There they rapidly proliferate and begin to produce pathogenetic macroglobulin. As a result, the formation of macroglobulinemia occurs.
predispose to the development of macroglobulinemia such factors:
- X-ray irradiation or radiation exposure;
- Chemical exposure factors, such as the use of certain drugs( antibiotics or gold salts) and the effects of industrial chemicals( inhalation or percutaneous ingestion), such as paint products, ethers, etc.;
- Factors of biological origin such as viruses, intestinal infections, tuberculosis lesions, stressful conditions or surgical treatment.
Until the end of the cause of the development of such pathogenic processes are not defined. Although there is a theory of the viral-genetic origin of macroglobulinemia, according to which, in the presence of special factors, viruses( about fifteen and a half) can enter the body. These viruses penetrate the immune defense and enter bone marrow immature cells, provoking their uncontrolled division.
A certain role in the development of pathology is played by the hereditary factor, because blood tumors develop more often in certain families or in individuals with structural chromosomal abnormalities.
Symptoms of
Until the appearance of a characteristic symptomatology in patients with , the disease of Maclongbulinemia Waldenstrom , weight loss, fatigue, persistent malaise and excessive weakness, hypertension and arthralgic manifestations are observed.
In addition, patients have such signs of pathology:
- Pathological lethargy;
- Periodic hearing impairment, prone to self-healing;
- Explicit retardation, sometimes reaching a loss of consciousness;
- Disturbance of limb mobility;
- The deposition of protein excesses in the tissues of internal organs, leading to the development of functional-articular disorders, problems with cardiac activity, increased language and its poor mobility, discomfort and soreness of various areas of the body caused by nerve damage.
A characteristic feature of Waldenstrom's macroglobulinemia is the tendency to gingival and nasal bleeding.
Much less common pathology is accompanied by subcutaneous hemorrhage. Such hemorrhagic symptoms are caused by impaired platelet gluing because of the high content of protein in the blood.
Complications of
Usually, macroglobulinemia first involves cardiovascular elements like venules, capillaries, etc.
As the intensity of the blood flow decreases, various eye retina lesions develop and renal function disorders due to urinary processes with urate deposits, vascular occlusion, etc.
In addition, macroglobulinemic processes are accompanied by a pathological decrease in immune defense and infections resulting from a decrease in the total organicezistentnosti( opportunistic infections).
With the further development of the disease, anemia and coma develop due to loss of consciousness due to blockage of vascular clearance in the brain arteries by surplus proteins.
Diagnosis
Diagnosis of the pathology is based on the general pattern of blood. The formation of adherent erythrocytes and the growth of ESR, the presence of unripe leukocytes, and the increased lymphocyte cell count all indicate the presence of Waldenstrom macroglobulinemia.
An additional procedure that supports this diagnosis is immunoelectrophoresis, which helps in detecting excessive M-immunoglobulin levels in the blood.
In addition, an additional sign that helps in diagnosis is an increase in the parameters of the liver or spleen, etc. Studies like MRI, ECG, X-ray diagnostics, ultrasound, SCT and other studies are carried out.
Treatment of
Disease The initial stages of development of macroglobulinemia are supposed to observe the tumor process. If there is an acceleration of the development of the tumor process or the growth of the medullary tumor, then treatment is indicated.
Therapy involves the use of several techniques:
- Chemotherapeutic treatment - involves the use of drugs that destroy malignant cells. This includes drugs, cytostatics, which have antitumor effect;
- Bone marrow transplantation - a similar technique is considered the only one capable of leading to a complete cure of the patient;
- Transfusion of red blood cells - a similar technique is necessary in case of development of pronounced anemia, when the patient has anemic coma or hemoglobin level is less than 70 g / l;
- Extracorporeal therapy - involves blood purification through specialized devices such as plasmapheresis, hemodialysis or hemosorption, etc.
Prognosis of life expectancy of patients and prevention of
In the presence of Waldenstrom's macroglobulinemia disease, life expectancy is usually of the order of 6-7 years, provided that the patient will be providedtimely and modern therapeutic care.
Unfortunately, there are no specific preventive measures to prevent the development of macroglobulinemia.