Polycythemia - a disease characterized by an increase in erythrocyte volume in the bloodstream.The disease can be caused by both primary and secondary impacts arising as a result of certain underlying causes.And primary and secondary polycythemia are quite terrible diseases, which can lead to serious consequences.So
primary or polycythemia vera appears when a tumor in the bone marrow of the substrate and increased production of red blood cells.Therefore, in advanced stages of this defeat is increased in size and replacing bone marrow all other substrates - the progenitors of future blood cells.
Secondary polycythemia occurs when a completely different situations, but is one of the leading general hypoxia (oxygen deficiency) organism.Thus, secondary polycythemia is indicative of certain pathological processes in the body, has emerged as a compensatory response.
Polycythemia vera is a disease with a highly tumor genesis.Fundamental to this disease is that the
polycythemia malignant course quite different and difficult to treat.This is due to the fact that it is practically impossible to work on the root cause of polycythemia vera - mutated stem cells with high mitotic activity (ability to divide).
striking and characteristic feature of polycythemia will pletorichesky syndrome.It is caused by a high content of red blood cells in line.This syndrome is characterized by a purple-red color of the skin with its strong itching.
In polycythemia vera, there are three main stages, which are arranged according to the activity of the process.The first stage - the stage of the swing.In this step, the first change will be formed in the bone marrow and form modified portions of hematopoiesis.The step height is almost impossible to detect clinically.In most cases, at this stage the diagnosis of polycythemia vera placed randomly, for example in studies of blood for the diagnosis of other diseases.
After the step height of the step of the clinical manifestations: here you will see all the clinical signs of the disease, a syndrome plethora, itchy skin, enlarged spleen.After the stage of clinical manifestations will be the final stage - anemic.When it will be revealed all the same clinical features, plus it will be added to the diagnostic symptoms of "devastation" of the bone marrow (due to constant bone marrow hyperplasia).
Importantly, polycythemia vera is a terrible disease because of its complications.The increased number of red blood cells and platelets will lead to increased thrombosis and thrombotic lesions of the body.In addition, increases the overall blood pressure, which may lead to persistent hypertension and hemorrhagic stroke, intracranial hemorrhage followed and death.
When polycythemia main manifestation is an increase in the bloodstream during those or other reasons, the number of red blood cells.Reasons for this arrangement will depend on the type of polycythemia.There are absolute and relative polycythemia type.
When absolute polycythemia is a direct increase in the volume of red blood cells in the bloodstream.For absolute polycythemia include polycythemia vera, polycythemia under hypoxic conditions, and obstructive lesions of the lungs, hypoxia associated with renal and adrenal glands.In all these states comes increased synthesis of red blood cells.
in polycythemia vera erythrocytes heavily synthesized hyperplastic tumor of the bone marrow sites, hypoxia response will cause an increase in the number of red blood cells and, in certain lesions of the kidney may increase the synthesis of erythropoietin - the main hormone responsible for triggering the formation of new red blood cells.
When the relative polycythemia, erythrocyte volume will increase due to reduced plasma volume.The normal plasma by approximately 5% more than the blood cells.With the loss of plasma this relation is violated, the plasma becomes smaller.The main paradox is that while the number of red blood cells relative polycythemia virtually unchanged - it remains within the normal range.But by reducing the blood plasma in the plasma ratio: forming elements, they become more - increase their "relative" number.
So to relative polycythemia include infectious diseases such as cholera, dysentery and salmonellosis.When they exhibit severe vomiting and diarrhea, which leads to a significant loss of body internal stores water including plasma.In addition to a reduction in plasma volume and the development of relative polycythemia can cause burns, as well as human exposure to high temperatures that cause sweating.
also need to pay special attention to the two most common causes of polycythemia: neoplastic lesions of bone marrow and the impact on the synthesis of red blood cells of hypoxia.
neoplastic lesions of bone marrow is fundamental for the development of primary or polycythemia vera.With this type of polycythemia is a mutation at the level of the genome of stem cell, and she begins to share uncontrollably, creating their new tumor clones.It is important to note that these pluripotent stem cells - this "alpha and omega" all future blood cells: red blood cells, white blood cells and platelets.These pluripotent cells are specific differentiation during its growth and form the three main areas of hematopoiesis in the number of formed elements: red blood cell, platelet and leukocyte.Then, from each of the germ gradually formed elements of the future are born.
But polycythemia pluripotent stem cells have a genetic defect, and synthesizes it exactly the same defective progenitor cells following areas of hematopoiesis.As a result, these cells are part of the hematopoietic and germs, as well as their progenitors strongly divided, creating a huge amount of formed elements.Thus, the formation of two pathological processes in polycythemia vera - exceeding the normal levels of red blood cells and simultaneous hyperplasia (increase in size) lesions hematopoiesis.
Effects of hypoxia on the body exerts on him a number of pathological phenomena, among which an important place is occupied by the development of secondary polycythemia.The cause of secondary polycythemia during hypoxia due to the fact that the body tries to compensate for the lack of oxygen in the body to synthesize new red blood cells.This occurs due to the effect of hypoxia on the kidney, during which the latter produces a special substance - erythropoietin.It erythropoietin triggers the differentiation of pluripotent stem cells in the reticulocytes (red blood cell progenitor) and further the formation of new red blood cells.Therefore, the effects of hypoxia on the blood can be traced to the following relationship: the greater will be its effect on the body, the more erythropoietin is synthesized by the kidneys, and the stronger will run red cell portion of the bone marrow to synthesize new red blood cells.
main and perhaps the most important feature of polycythemia is the so-called "syndrome of plethora."This syndrome is caused by an increase in the volume of blood cells and overall congestion.
basis pletoricheskogo syndrome will make patients' complaints and violations, which can be determined by objective research.
Among the main indicators of patient complaints pletoricheskogo syndrome are chronic headaches that will alternate with dizziness.In addition pletorichesky syndrome will always be accompanied by complaints of patients itching.Its appearance is due to the fact that when there is a massive polycythemia synthesis of mast cells special substances - histamines and prostaglandins, which are acting on histamine receptors, will lead to the emergence of a strong, sometimes unbearable itching.By the way, one of the classic and typical for polycythemia will be the special character of pruritus - it can increase several times, after skin contact with water (for bathing, showering or even by simple washing).But it is important to know that the plethora - a sign of a purely polycythemia vera.In secondary polycythemia color of the skin does not undergo such a large change.
addition to the above complaints, patients will also indicate changes in the hands.These changes are called rodonalgia.The skin of hands is characteristic of the "red-cyanotic" color.In addition discoloration of the hands and fingers will be accompanied by severe pain in the affected areas, which will occur at each contact with any surface.The reason for this is the same state as when pletoricheskom skin itch - production of high amounts of histamine.
Objectively, we can determine the presence of patients with polycythemia specific coloring - skin are bluish-red, sometimes cherry.There will also be revealed significant changes in the cardiovascular system.The most characteristic of them are: excessive blood pressure and thrombosis.A characteristic feature of changes in blood pressure when polycythemia is an increase in systolic blood pressure over 200 mm Hg.
second syndrome when polycythemia will myeloproliferative syndrome.This symptom is more typical is for real or primary polycythemia.This syndrome is accompanied by an enlarged spleen or liver.His main reason - is that the spleen in the body acts as a so-called "depot" or "warehouse" for red blood cells.The normal red blood cells in the spleen, ends its life cycle are destroyed.But since the number of red blood cells polycythemia can sometimes exceed the rate of ten times, and the number of red blood cells accumulate in the spleen is enormous.As a result of spleen tissue hyperplasia occurs and its increase.To increase the spleen syndrome mieloproliferatsii also added complaints of patients of weakness, fatigue, and pain in the long bones in the left upper quadrant.Painful symptoms are explained by the growth of just the changed portions of hematopoietic tumors and hyperplastic spleen.
addition for any polycythemia may see symptoms that are associated with increased blood viscosity.They include small and large thrombosis, stroke and heart attacks, as well as the separation of clot and subsequent development of pulmonary embolism.
But apart from the above-described symptoms of polycythemia, directly related to the pathogenesis of the disease, namely the increase in the number of red blood cells in the bloodstream, and following this state of change, there are also the symptoms - symptoms of major diseases that have led to the development of secondary polycythemia.These symptoms include cyanosis (cyanosis acrocyanosis and common) for the root causes of secondary polycythemia on the part of the respiratory system (mostly for its appearance can cause chronic obstructive lesions of the pulmonary system) and total exposure of the body hypoxia.There may also be symptoms of a renal dysfunction or tumor lesions, which can also cause secondary polycythemia.
In addition, we must not forget about the impact of infectious agents to the primary mechanism of polycythemia.Key features in infectious symptoms caused by secondary polycythemia are profuse diarrhea and vomiting, which will lead to a sharp decrease in plasma volume, and therefore non-specific increase in the number of red blood cells.
first signs of polycythemia may also be observed in neonates.Polycythemia newborn occurs as the body's response to the child he underwent intrauterine hypoxia, which could develop due to placental insufficiency.In response, the body baby, trying to correct hypoxia, begins to synthesize an increased number of red blood cells.The same lack of oxygen as a trigger factor of neonatal hypoxia can be observed in the presence of a newborn "blue" congenital heart or lung disease.
Also due to respiratory polycythemia in newborns as well as adults, can develop and polycythemia vera.Especially strong this risk may be exposed twins.
The disease occurs in the first weeks of life of the newborn and the first signs of it will be a significant increase in hematocrit (60%) and the increase in hemoglobin is 22 times.
in neonatal polycythemia there are several stages of clinical course: the initial stage, the stage of proliferation and the stage of exhaustion.
At the initial stage of polycythemia practically does not manifest itself and develop without any clinical manifestations.Furthermore, to determine the presence of polycythemia child at this stage can only examining the peripheral blood: hematocrit, hemoglobin and red blood cells.
Stage proliferation is a much more vivid clinic.At this stage, the child found enlarged liver and spleen.Develop pletoricheskie effects: skin are typical "pletoricheski red" hue, anxiety baby when touched to the skin.It will be added pletoricheskomu syndrome thrombosis.The analysis will change in the number of platelets, red blood cells and leukocyte changes.Also can increase the performance of all blood cells - this phenomenon is called panmieloza.
in exhaustion at the child still be signs of an enlarged spleen and liver, will be significant weight loss, asthenia and exhaustion.
These clinical changes are very difficult for the newborn, and can lead to irreversible changes and subsequent death.Furthermore polycythemia newborn can cause sclerosis processes in bone marrow have been due to the constant proliferation of tumor cells in the bone marrow is displaced normally operating hematopoietic tissue and replacement by connective.In addition, such a phenomenon can disrupt production of certain types of white blood cells responsible for the immune defenses of the child.As a result, the newborn may develop severe bacterial infections, which are the cause of their death.
treatment of Polycythemia
for proper treatment of polycythemia is important to determine the root cause, which was the trigger factor of the disease.That's the difference in the impact on the main trigger factor of polycythemia will be fundamental in the treatment regimen.For example, when the secondary polycythemia involved removing its underlying cause, and for polycythaemia vera try to influence tumor cell arrest and effects of increased production of cells - blood cells.
polycythemia is quite difficult to treat.Hard enough to affect tumor cells and stop its activity.Also fundamental in the treatment of polycythemia appointment aimed at the suppression of tumor cell metabolism is age.