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[edit | edit source]
- 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[edit | edit source]
- 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.[edit | edit source]
- 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[edit | edit source]
- Medical venipuncture , erythrocytapheresis (more expensive, exceptionally),
- IFN-α ,
- hydroxyurea,
- anagrelide + therapeutic venipuncture in patients resistant to IFN + hydroxyurea,
- treatment of pruritus + hyperuricemia .
Prognosis[edit | edit source]
- Average survival 15 years,
- cause of death: thrombosis, acute leukemia , other cancer, bleeding , etc.[1][2]
Summary video[edit | edit source]
Links[edit | edit source]
Reference[edit | edit source]
Related articles[edit | edit source]
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
digestive problems, frequent
Gastroduodenal ulcer disease
,
frequent bleeding / conversely arterial and venous thrombotic events, incl. coronary artery obliteration,
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.[1]
[2]
Summary video
Links
Reference
- ↑ NEČAS, Emanuel. Patologická fyziologie orgánových systémů : Část I. 2. edition. V Praze : Karolinum, 2009. 379 pp. ISBN 978-80-246-1711-4.
- ↑ DÍTĚ, P.. Vnitřní lékařství. 2. edition. Praha : Galén, 2007. ISBN 978-80-7262-496-6.
