Erythrocytosis is a condition of increased red cell mass and has a very heterogeneous etiology. Patients may be asymptomatic or have symptoms and signs of increased blood viscosity. A diagnostic algorithm is applied to confirm the precise etiology necessary for appropriate treatment. In the first step, we have to confirm absolute erythrocytosis with hemoglobin (Hb) > 185 g/L and/or hematocrit (Ht) > 0.52 for men and Hb > 165 g/L and/or Ht > 0.48 for women. In the second step, we exclude polycythaemia vera and also search for secondary acquired causes such as lung-, heart- and kidney diseases and tumours with exogenous erythropoietin secretion. It is worth mentioning that according to the WHO guidelines, the diagnosis of polycythaemia vera in patients with an appropriate clinical picture is indicated at lower values, more precisely, Hb > 165 g/L or Ht > 0.49 for men and Hb > 160 g/L or Ht > 0.48 for women. In the third step, we refer patients without a known cause of erythrocytosis for genetic testing to identify congenital erythrocytosis. People in whom we have excluded polycythaemia vera, secondary acquired and congenital erythrocytosis, have idiopathic erythrocytosis. Treatment depends on the cause of the erythrocytosis. Most commonly, it includes acetylsalicylic acid and venipunctures.
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