polycythemia - a blood disease from the group of chronic leukemia characterized by tumor proliferation (growth), mainly red blood cells.Therefore, this disease is another name eritremii (from the Greek words "red" and "blood").
The cause of polycythemia is unknown.It is assumed that in this disease initially disturbed regulation of the process of formation of red blood cells.
In the modern theory of blood, all human blood cells have a single progenitor cells.Sharing and breeding, the descendants of stem cells are becoming increasingly specific features and eventually become red blood cells, white blood cells or platelets.When eritremii balance of blood cell changes, begins uncontrolled excessive formation of red blood cells.However, other cells (leukocytes and platelets) are also formed in excess, but not as prono
As a result, a person's blood comes increased number of red blood cells is not due to external causes.This erythremia different from polycythemia, which are the body's response to external factors (eg, lack of oxygen in the air).
increase in the number of red blood cells and platelet dysfunction leads to increased thrombosis.
With the progression of the disease can occur so-called myeloid metaplasia, which is characterized by inhibition of hematopoiesis germs with the development of anemia.
Appearance patient with polycythemia vera quite typical.Most often it is a middle-aged or older, overweight.Face flushed, injected sclera.The lips and tongue are characteristic cherry color.These external symptoms are called "erythrose".
patients revealed signs of dysfunction of the central nervous system.There have been complaints of headache, dizziness, tinnitus.Frequent fainting and nosebleeds.Sometimes the patient's state of health is deteriorating so that it can not do any mental work.Worried about memory loss and attention, fatigue, irritability.
Patients often point to chest pain.However, these feelings are often caused by soreness of the sternum as a result of increased blood supply to her fabric.However, in such patients at high risk of thrombosis, including coronary artery, the development of angina pectoris and myocardial infarction.
Thrombotic complications may cause thrombophlebitis, deep vein thrombosis tibia mesenteric veins to the development of the related symptoms.It is possible, and the occurrence of cerebrovascular events.
patients with erythema often diagnosed a stomach ulcer that is associated with the violation of the nervous regulation of an organism.Occasionally developing hypertension (this is not a characteristic symptom of polycythemia).
Along with thrombosis often marked hemorrhagic syndrome associated with a tendency to bleed.Concerned not only bleeding from the nose, but also hemorrhoids from esophageal varices, and bleeding gums.There have been and bruising easily formed ecchymosis (bruising).
About half of patients have an intense itching after taking a hot bath, a characteristic symptom of eritremii.Some patients experience a burning pain in the fingertips, which is also characteristic of polycythemia vera.It may be broken tactile and pain sensitivity.
In most patients, increased spleen, which can manifest itself as a feeling of heaviness in the left upper quadrant, so the feeling of satiation excessive eating.
How is the disease?Current eritremii may be relatively benign, when patients live many years without serious complications.In some cases, a few years after the first symptoms of the disease have serious thrombosis of cerebral vessels or vessels of the abdominal cavity, leading to death.
objective picture of the first stage of giving blood.Suspected erythremia needed if the number of red blood cells is greater than 5.7 * 1012 / L for men and more than 5.2 * 1012 / L in women.The hemoglobin level while above 177 g / l for men and 172 g / L in women.Diagnosis of the disease is based on specific criteria.An essential component of the diagnostic research is a biopsy of the ilium.
Treatment of polycythemia vera is conducted mostly on an outpatient basis.Indications for hospitalization - an expression of severe illness, reducing the number of leukocytes and platelets after treatment with cytostatics, the need to puncture the bone marrow or spleen, trepanobiopsy ilium.The patient must be hospitalized if the planned surgery, even minor (eg, tooth extraction).
Therapeutic program includes the following areas:
This is the main method of treatment in people younger than 50 years.When removing from the body of the circulating blood is discharged bloodstream, facilitated by itchy skin, decreases the probability of thrombotic complications.
as an independent method of treatment used in bloodletting benign polycythemia, and childbearing and premenopausal.If after a course of chemotherapy relapsed eritremii can also be assigned to the bloodletting.They must be carried out before pronounced reduction in hemoglobin level (not higher than 150 g / l).
procedure is usually performed in a clinic.Within one session is withdrawn from 350 to 500 ml of blood.Sessions bloodletting repeated after 2 days to achieve the desired performance.In the future, 1 control blood every two months.
a result of bleeding in the body decreases the iron content.Most often, patients tolerate it well.However, sometimes there is a weakness, hair loss, severe iron deficiency anemia.In such a case should be given iron supplements in combination with cytotoxic drugs.
Bleeding not appointed in their small and short-term effects, at the expressed signs of iron deficiency.
During this procedure, the vascular bed of the patient take 1 - 1.4 liters of blood.From this by means of special equipment removed erythrocytes.The remaining plasma was adjusted to the original volume with physiological saline and infused into the venous system.Eritrotsitaferez an alternative to bloodletting.Courses give the effect of this treatment for 1-2 years.
In severe cases of illness, with the ineffectiveness of bleeding in individuals older than 50 years are appointed by cytotoxic drugs.They inhibit the multiplication of cells in the bone marrow.This reduces the number of all blood cells, including red blood cells.In the treatment with cytostatics regularly conduct blood tests which monitor the efficacy and safety of treatment.
most commonly used alkylating cytotoxic agents and anti-metabolites.The radioactive phosphorus 32P is used less frequently, he shows, mainly the elderly.
When thrombosis prescribed antiplatelet agents, heparin.In acute thrombophlebitis conducted local treatment: cooling foot ice pack on the first day, and then heparin ointment Vishnevsky ointment.
When expressed bleeding appointed aminocaproic acid, fresh frozen plasma, hemostatic sponge locally.
rodonalgia (pain in the fingertips, the soles) treated with NSAIDs (indomethacin, Voltaren).Can be assigned to antiplatelet and heparin.
When stroke, hypertension, gastric ulcer medication used appropriate scheme.For the treatment of pruritus used antihistamines.Sometimes it turns out to be an effective cimetidine (H2-receptor blocker).
Indications for removal of the spleen when eritremii: