Polycythemia Vera
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-PV |
|---|---|
| Type | Disease |
| Aliases | ErythremiaPVPolycythemia rubra veraСправжня поліцитеміяХвороба Вакеза |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-ESMO-MPN-2015 SRC-NCCN-MPN-2025 SRC-RESPONSE-VANNUCCHI-2015 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9950/3 |
|---|---|
| icd_o_3_topography | ["C42.1"] |
| icd_10 | D45 |
| who_classification | Polycythaemia vera (PV) — Ph-negative myeloproliferative neoplasm (per WHO 5th ed.) |
Notes
Authored as a separate disease from ET (rather than a single combined PV-ET YAML). Rationale (per myeloid expansion commit message): JAK2 V617F prevalence is essentially universal in PV (~95%) but only ~50% in ET; thrombotic-risk drivers differ (Hct + RBC mass in PV vs platelet count + JAK2 + age in ET); 1L cytoreduction shares HU but PV adds mandatory phlebotomy + low-dose ASA baseline AND ruxolitinib has a PV indication (RESPONSE) distinct from MF. ET YAML reuses overlapping RFs but has its own algorithm. STUB — requires clinical co-lead signoff before publication. Myeloid expansion (heme).
Used By
Actionability
BMA-JAK2-V617F-PV- JAK2 V617F is the defining driver of polycythemia vera (~95%) and a WHO 2022 / ICC 2022 m...
Algorithms
ALGO-PV-1L- ALGO-PV-1LALGO-PV-2L- ALGO-PV-2L
Disease
Indication
IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-OBSERVATION- IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-OBSERVATIONIND-MPN-SECONDARY-LEUKEMIA-PREVENTION-SURVEILLANCE- IND-MPN-SECONDARY-LEUKEMIA-PREVENTION-SURVEILLANCEIND-PV-1L-HU- IND-PV-1L-HUIND-PV-1L-PHLEBOTOMY-ASA- IND-PV-1L-PHLEBOTOMY-ASAIND-PV-1L-ROPEGINTERFERON- IND-PV-1L-ROPEGINTERFERONIND-PV-2L-RUXOLITINIB- IND-PV-2L-RUXOLITINIBIND-PV-ANAGRELIDE-CONTINUOUS- IND-PV-ANAGRELIDE-CONTINUOUSIND-PV-PREGNANCY-PEG-IFN- IND-PV-PREGNANCY-PEG-IFN
Questionnaires
QUEST-PV-1L-STUB- Polycythemia Vera — first line
Red flag
RF-MPN-SECONDARY-LEUKEMIA-PREVENTION- Myeloproliferative neoplasm (polycythemia vera [PV], essential thrombocythemia [ET], or p...RF-PV-ET-FRAILTY-AGE- PV or ET patient elderly or frail (age ≥80, ECOG ≥3, multiple comorbidities, life expecta...RF-PV-ET-HIGH-THROMBOSIS-RISK- PV or ET high-risk for thrombosis: age >60 OR prior arterial / venous thrombosis OR (ET o...RF-PV-ET-INFECTION-SCREENING- PV / ET patient with positive HBV serology, latent TB, or chronic infection — primarily r...RF-PV-ET-ORGAN-DYSFUNCTION- PV or ET patient with organ dysfunction limiting cytoreductive choice: severe renal impai...RF-PV-ET-PREGNANCY-OR-PLANNING- PV or ET patient pregnant or planning pregnancy — HU and anagrelide contraindicated; swit...RF-PV-HU-RESISTANCE-INTOLERANCE- PV resistant or intolerant to hydroxyurea per ELN criteria: persistent need for phlebotom...
Sources
SRC-ESMO-MPN-2015- Philadelphia-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidel...SRC-PROUD-PV-GISSLINGER-2020- Ropeginterferon alfa-2b versus standard therapy for polycythaemia vera (PROUD-PV and CONT...SRC-RESPONSE-VANNUCCHI-2015- Ruxolitinib versus standard therapy for the treatment of polycythemia vera