Polycythaemia vera

Polycythaemia vera blood count

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.

Links

Related articles

• Myeloproliferative disease
• Secondary polyglobulia
• Erythrocytosis