Erythremia ( polycythemia vera )
Oncology / / May 14, 2016
Erythremia is a myeloproliferative disease characterized by a significant increase in the formation of red blood cells in the bone marrow and increasing their numbers in the circulating blood, which is often accompanied by excessive formation of granulocytes and platelets.The maximum incidence occurs between the ages of 50-60 years, but the disease can develop at any age, more common in men.The absolute incidence of the disease is 0.4-0.5 new cases per 100 000 people per year.
eritremii etiology remains unknown.The experimental conditions very similar syndrome was observed in mice infected with Freund's, but extrapolation of these results to the human is not feasible.Rarely observed familial incidence and increased incidence of disease among the Jews eritremii may indicate genetic correlations.
With pathogenetic point of view, the presence of leukocytosis and thrombocytosis but excessive red blood cell assumes a pathological lesion of stem cells of hematopoiesis.The reason of its differenti
clinical picture is mainly characterized by changes associated with an increase in the total blood volume.Rich cellular elements dark red bone marrow also applies to those areas that are usually occupied by fatty bone marrow.The percentage of mature erythroblasts can be increased together with the young differentiating granulocytes.The spleen is enlarged, filled with blood, it often develop heart attacks.The viscosity of the blood is increased, and the rate of blood flow is greatly reduced.The resulting stagnation of visceral with concomitant thrombocytosis causes intravascular thrombosis and thromboembolic disorders.A more progressive forms eritremii tendency to bone marrow changes with symptoms of extramedullary hematopoiesis in a significantly enlarged spleen and liver.
Clinical data and may be associated with different disease progression.During eritremii can recognize three stages;starting with a large volume of red blood cells, an intermediate stage myelofibrosis with compensated and decompensated end with myelofibrosis or acute leukemic syndrome.
initial stage marked with a large number of red blood cells, with an increased hemoglobin and hematocrit can last for many years.Symptoms and signs are mainly associated with high blood volume.Patients have the form congested develops redness of the mucous membranes, and they may complain of headache, heaviness in the head, inability to concentrate, fatigue, malaise, loss of health and energy.Characterized by the development of pruritus with scratching, especially after a hot shower or bath.Myocardial spleen - pain in it.There may be symptoms related to arterial, coronary, peripheral and cerebral insufficiency, hypertension, venous thrombosis and thromboembolism, increased abdominal organs.It is noted increased activity of bone marrow cells and megakaryocytes increase can be pronounced.Alkaline phosphatase activity in leukocytes increased.At this stage, very typical indicator is the decrease in ESR.With eritremii usually associated hyperuricemia and hyperuricosuria.
Under compensated myelofibrosis splenomegaly as the main area of extramedullary hematopoiesis may be expressed together with the enlargement of the liver.The formation of red blood cells is reduced, and the patient gradually disappear complaints associated with fluid overload, but malaise, fatigue and weakness persist.The bone marrow cells observed decrease in the number and in the peripheral blood of erythroblasts and found megakariotipicheskie fragments or abnormal platelets.The entire spectrum of young immature granulocytes with high leukocytosis may give a picture of the chronic phase of chronic myelocytic leukemia.This stage can last for months or years.
last stage can be characterized by a syndrome of decompensated myelofibrosis or acute leukemia.Decompensation hematopoiesis manifested in progressive anemia, bleeding tendencies to, infection, excessive fatigue and malaise of the patient.Acute leukemia syndrome is very similar to other manifestations of acute leukemia, especially with blast phase chronic myeloid leukemia, and it is considerably faster due to insufficient recovery of normal hematopoiesis due to fibrotic primary malignancy of the bone marrow.
Differential diagnosis in the early stages is very difficult, especially if it relates to a secondary eritremii normally associated with increased secretion of erythropoietin.This happens during hypoxia, kidney stones, kidney tumors, hemangioblastoma of the cerebellum, uterine fibroid disease, lung cancer and hepatoma.In these diseases there is no itching, occasionally there splenomegaly, white blood cell count is usually normal, a normal platelet count, leukocyte alkaline phosphatase activity as normal.In advanced stages of the disease during the previous eritremii can help correct diagnostic orientation.