Suspect-and-test checklist
Polycythemia vera
JAK2-mutated blood cancer. Start from a sustained high hemoglobin or hematocrit, an unusual clot or itch after water.
ICD-10-CMD45Trial Friend for Clinicians
1Red flags present
Blood count
Itch, hands, feet and vision
Clots
Examination and history
2Already labeled as
3Family to test
4Testing, in the order the sources describe
- Repeat blood count and review earlier countsSeparates longstanding from acquired erythrocytosis; sustained is typically 3 months or longer.Result
- History for secondary causesSmoking, heart and lung disease, sleep apnea, altitude, tumors, androgens, SGLT2 inhibitors.Result
- JAK2 V617F on peripheral bloodFirst step per Mayo Clinic and Italian authors. ICC advises assays with sensitivity under 1%.Result
- Serum erythropoietinSubnormal level is the minor criterion. Normal in 17% of patients with PV.Result
- JAK2 exon 12 (exons 12 to 15)If V617F is negative and PV is still suspected; other JAK2 mutations make up about 3%.Result
- WHO or ICC criteriaAll 3 major, or the first 2 major plus subnormal erythropoietin.Result
- Bone marrow biopsyMajor criterion. May be omitted above 18.5g/dL (men) or 16.5g/dL (women) with JAK2; WHO 2016 also asks for low erythropoietin.Result
- JAK2 V617F after a splanchnic clotBaveno VII: all adults with primary splanchnic vein thrombosis.Result
- Oximetry, p50 and hereditary panelFor JAK2-negative erythrocytosis with no cause found.Result
5Results the sources say do not rule it out
- Negative JAK2 V617F. Other JAK2 mutations, including exon 12, account for about 3%.
- Normal erythropoietin. Subnormal in 81%, normal in 17%.
- Hemoglobin below the threshold with iron deficiency, a large spleen or a rise from baseline.
- A sleep apnea, smoking or testosterone label. Sources still put JAK2 screening first.
- Another clot risk factor or an evident stroke cause.
- Positive JAK2 V617F alone. Also found in ET, myelofibrosis and 3.1% of one general population.
6Specialists the sources name
Diagnosis code: D45 Polycythemia veraCodes patients often carry before diagnosis are listed in the online guide.
Full guide and sourcesEvery point here is sourced in the online guide, which also has a patient note to print.
Trial Friend, trialfriend.com/for-clinicians. Key sources: ICC 2022 criteria (Gianelli 2023); WHO 5th edition (Khoury 2022); WHO 2016 criteria summary (Barbui 2018); JAMA review 2025; International PV study (Tefferi 2013); Tefferi, Vannucchi and Barbui 2021; all 39 with links in the online guide. Checked November 12, 2026. A reference checklist for licensed clinicians that summarizes published sources. It is not a diagnostic tool, a score or a protocol, and it does not replace clinical judgment, local practice or the prescribing information. Not coding advice. Trial Friend never receives anything written or typed on this sheet.