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Start free with EleplanPolycythemia vera
ORPHA:729Disease
Also called Acquired primary erythrocytosis · Osler-Vaquez disease · PV · Polycythemia rubra vera · Vaquez disease
What it is
A rare acquired myeloproliferative neoplasm characterized by an elevated absolute red blood cell mass (RBCM) caused by uncontrolled red blood cell production, frequently associated with uncontrolled white blood cell and platelet production.
Key facts
- Prevalence
- 1-5 / 10 000 (Europe)
- Age of onset
- All ages
- Inheritance
- Not applicable
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
16These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Gene
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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