What is polycythemia

At polycythemia (Thesaurus synonyma: emotional polyglobulia; acquired polyglobulia; acquired polycythemia; erythrocytosis; Forssell syndrome; high mountain polyglobulia; high altitude polyglobulia; high altitude polyglobulia; hypertensive polyglobulinia; hypoxemic polyglobulia; polyglobarogenemia; Polyglobulia; Polyglobulia due to stay at high altitude; Polyglobulia due to erythropoietin; Polyglobulia due to haemoconcentration; Polycythemia due to stay at high altitude; Polycythemia due to stress; Pseudopolyglobulia; Pseudopolycythemia; Relative polyglobulia; Secondary polyglobulia; Renal polyglobulia; Secondary erythromatemia; Polyglobulia; Secondary erythromatemia; Polyglobulia; Vaquez-Osler disease; Vaquez-Osler syndrome; ICD-10-GM D45: polycythemia vera; ICD-10-GM D75.1: secondary polyglobulia) it is an erythrocytosis (Increase in erythrocytes, i.e. the red blood cells in the blood).

Polycythemia can be congenital or acquired.

A distinction can be made between several forms:

  • Primary polycythemiaPolycythemia vera(PV), benign familial polycythemia; Disorder of the erythropoietic stem cell; autonomous proliferation of the three rows of cells
  • Secondary polycythemia (Polyglobulie) - isolates increased erythrocyte count with normal plasma volume, e.g. B .:
    • reactive stimulation of erythropoiesis (blood formation) in hypoxia (insufficient oxygen supply to the tissue; e.g. heart defects with right-left shunt, chronic lung diseases, staying at high altitudes)
    • renal polyglobules
    • hypertensive (high blood pressure) polyglobulia
  • Relative polycythemia (Pseudopolyglobulia) - by reducing the plasma volume with isolated borderline or moderate increase in the number of erythrocytes (stress erythrocytosis)

Polycythemia vera (PV) is one of the myeloproliferative neoplasms(MPN) (formerly: chronic myeloproliferative diseases (CMPE)). These are diseases in which there is an increase in all three rows of blood cells (especially erythrocytes, but also thrombocytes (blood platelets) and leukocytes / white blood cells) in the blood.

PV is the most common of the myeloproliferative neoplasms. The following other diseases are among the myeloproliferative neoplasms:

  • Chronic myeloid leukemia (CML)
  • Essential Thrombocythemia (ET) - chronic myeloproliferative disease (CMPE, CMPN), which is characterized by a chronic increase in platelets (platelets)
  • Osteomyelofibrosis (OMF; synonym: osteomyelosclerosis, PMS) - myeloproliferative syndrome; is a progressive disease of the bone marrow.

It will two clinical stages of Polycythemia vera differentiated:

  1. a chronic phase:
    • increased erythrocyte production and erythrocytosis (increase in the number of erythrocytes), which can last up to 20 years and
  2. a progressive late phasee (formerly referred to as the "spent phase"):
    • secondary marrow fibrosis with extramedullary hematopoiesis (blood formation outside the bone marrow) and increasing splenomegaly (enlargement of the spleen; up to 25% of patients) and / or due to the transition to:
      • myelodysplasia (group of diseases of the bone marrow in which blood formation does not come from healthy but from genetically modified cells of origin (stem cells)) or acute myeloid leukemia (approx. 10% of patients)

Gender ratio: Men to women is 2: 1 (with regard to polycythemia vera).

Frequency peak: The maximum occurrence of polycythemia vera is around the age of 60.
The median age of patients at diagnosis is 60 to 65 years.

The Incidence (Frequency of new diseases) of polycythemia vera is around 0.7-2.6 diseases per 100,000 inhabitants per year.

Course and prognosis: If the disease is not recognized, survival time is only a few years. So far there is no cure for polycythemia. However, adequate therapy can slow the progression (progression) of the disease. Life expectancy is then not significantly restricted.


     
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