Polycythaemia vera

Polycythaemia vera (PV, primary polycythaemia, Vaquez's disease) is a disease with a high number of erythrocytes + high concentration of Hb → increases blood viscosity + arterial thrombotisation (cause of death is thrombosis of coronary + cerebral arteries ).

Pathogenesis

 * Clonal proliferation of a pluripotent hematopoietic stem cell, kt. differentiates mainly into the erythrocyte line;
 * increased sensitivity of BFU-E progenitors to the effects of erythropoietin;
 * the possibility of differentiation into erythroid precursors even without erythropoietin;
 * there is also a slight increase in bb. other rows.

Clinical picture

 * Headache, dizziness , etc.,
 * digestive problems, frequent Gastroduodenal ulcer disease ,
 * pruritus,
 * frequent bleeding / conversely arterial and venous thrombotic events, incl. coronary artery obliteration,
 * Arthritis uratica (gout),
 * brownishness, cyanosis ,
 * splenomegaly (in advanced stages up to the pelvis).

Diagnostics a diff. dg.

 * Increased value of Hb + HTK (50-70%),
 * can be masked if plasma is also increased;


 * there may be leukocytosis + a slight shift to the left,
 * often also thrombocytosis,
 * blood oxygen saturation < 92% can be the cause of secondary polyglobulia ,
 * low ferritin values ​​more common in primary polycythemia than in secondary polyglobulia; concentration of vitamin B12 + its binding capacity increased, conc. folate reduced,
 * in PV, the concentration of endogenous erythropoetin is reduced,
 * the histological height is essential. bone marrow (PV image different from reactive polyglobuli) + molecular-biological examination,
 * dg. PV rests hl. in the detection of general signs of myeloproliferative disease, in the case of embarrassment to exclude a secondary cause (difficult),
 * frequent thrombotic/bleeding events (in patients with secondary polyglobulia few).

Therapy

 * Medical venipuncture, erythrocytapheresis (more expensive, exceptionally),
 * IFN-α ,
 * hydroxyurea,
 * anagrelide + therapeutic venipuncture in patients resistant to IFN + hydroxyurea,
 * treatment of pruritus + hyperuricemia.

Prognosis

 * Average survival 15 years,
 * cause of death: thrombosis, acute leukemia, other cancer, bleeding , etc.

Related articles

 * Myeloproliferative disease
 * Secondary polyglobulia
 * Erythrocytosis