Polycythemia (erythrocytosis) - a group of pathological conditions characterized by an increase in the number of red blood cells (regardless of the number of white blood cells, platelets).In this article we will discuss the signs and symptoms of polycythemia basic humans.
symptoms of polycythemia
Symptoms of polycythemia vera (polycythemia vera) - chronic leukemia with damage to the progenitor cells myelopoiesis level characteristic of unlimited proliferation of tumor of the cells retain the ability to differentiate 4 germs, especially the red.
incidence of disease in the population - 0.6 per 10,000 population.Men suffer more often (1.2: 1).The average age of patients 60 years of age.
etiology of the disease is unknown.
pathogenesis of symptoms caused enhance erythropoiesis, erythrocytosis in the peripheral blood with the development of the secondary flow and coagulation disorders, myeloid metaplasia of the spleen and liver, the final exhaustion o
erythrocytosis peripheral blood leads to increased blood viscosity and Ht, reduced blood flow and a decrease in tissue oxygenation.Increased cardiac output (compensatory mechanism in the tissue hypoxia).Stagnation of blood in the vessels (decreased tissue perfusion) can provoke thrombosis.
symptoms of polycythemia
disease begins gradually, with redness of the skin, there is a symptom of a heaviness in the head, there is hypertension, increases the size of the spleen, there is itching, worse after taking a bath.
Sometimes the first symptoms of the disease may be peripheral thrombosis, myocardial infarction, pulmonary embolism.
signs of polycythemia
There are three stages of polycythemia vera:
signs of the first stage of polycythemia
first stage (initial) is characterized by mild erythrocytosis blood panmielozom in red bone marrow.Vascular and visceral complications are rare.Spleen increased somewhat, but usually palpate it is not possible (due to an increase in the spleen increased sequestration in her platelet and red blood cells).The duration of the first step may be more than 5 years.
Signs of the second stage of polycythemia
Unfolded (proliferative) phase is characterized by a pronounced plethora, hepatosplenomegaly myeloid metaplasia by these bodies, recurrent thrombosis, patients are depleted.Blood results show polycythemia, thrombocytosis and polycythemia or panmieloz, neytrofilёz shift leukocyte formula to the left, increasing the content of basophils.In the red bone marrow hyperplasia show a total of three germ hematopoiesis with expressed megakariotsitozom, possible retikulinovy and focal collagen myelofibrosis.The increased serum concentration of uric acid.
Signs of the third stage of polycythemia
third stage - anemic (exhaustion).The liver and spleen are enlarged, they exhibit myeloid metaplasia.In the blood increases pancytopenia, in the red bone marrow myelofibrosis progresses.
Laboratory and instrumental investigations polycythemia
general analysis of blood
leukocytosis, thrombocytosis, increased alkaline phosphatase activity of white blood cells and an increase in the concentration of vitamin Bi2 in serum.
Proliferative symptoms - hyperplasia three hematopoiesis germs, pathological megakaryocytes, no iron deposits (more than 95% of cases), the signs of incipient fibrosis (increased reticulin content).
Stage of exhaustion - ineffective hematopoiesis, hematopoietic tissue fibrosis, accumulation of megakaryocytes.
studies necessary for differential diagnosis:
- biopsy of the bone marrow, designed to reveal pancytopenia without fibrosis.
- concentration of erythropoietin is not changed.
- acid-base balance of blood (pH, p02) is not violated.
- Cytogenetic study to exclude the presence of "Philadelphia" chromosome.
- ultrasound (CT indication) of the internal organs in order to detect eritropoetinsekretiruyuschih tumors (in the adrenal glands, kidneys, etc.).
Differential diagnosis of polycythemia
Erythremia is not the only cause of erythrocytosis.Eritremii must be differentiated with absolute erythrocytosis secondary, secondary erythrocytosis relative to exclude primary polycythemia.
symptoms secondary absolute erythrocytosis associated with increased production of erythropoietin, which contributes to generalized tissue hypoxia, such as arterial hypoxemia due to chronic obstructive lung diseases, congenital "blue" heart defects, arteriovenous anastomoses, the syndrome of Pickwick or without arterial hypoxemia with hemoglobinopathieswith high affinity to oxygen deficiency in the 2,3-diphosphoglycerate erythrocytes.The cause of the symptom of increased synthesis of erythropoietin may arise eritropoetinsinteziruyuschie tumor (kidney cancer gipernefroidny, hemangioblastoma, hepatoma, uterine fibroids, tumors and cerebral cortical layer of the adrenal glands), cyst adenoma and pituitary, masculinizing ovarian tumors.Local ischemia of kidneys (cysts, hydronephrosis, renal artery stenosis) also leads to polycythemia.
secondary symptoms relative erythrocytosis - gemokontsentratsionnye, such as Gaysbёka syndrome, stress polycythemia.
be understood family nemieloproliferativnye disease under primary erythrocytosis.
need for radiological diagnostics measurement of the mass of circulating red blood cells (by radioactive chromium), and sometimes also in circulating plasma (serum albumin using labeled 1311).
When a normal mass of circulating red blood cells and decreased plasma volume relative diagnosed with polycythemia [Ht increased due to reduced plasma volume per body weight (with unmodified red blood cell count).