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Polycythemia Vera

A chronic myeloproliferative blood disorder characterized by excessive red blood cell production in the bone marrow.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Polycythemia Vera?

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by uncontrolled red blood cell (and usually leukocyte and platelet) production by the bone marrow due to a JAK2 kinase mutation (most commonly V617F). Its incidence is 2-3 per 100,000 per year; the average age at diagnosis is around 60.

Blood thickening significantly increases thrombosis risk: deep vein thrombosis, pulmonary embolism, Budd-Chiari syndrome, and stroke are the most common thrombotic complications. Paradoxically, bleeding may also accompany. There is a long-term risk of transformation to myelofibrosis or acute leukemia.

Diagnosis is based on WHO criteria: elevated hemoglobin, hypercellular bone marrow biopsy findings, and JAK2 mutation. Suppressed erythropoietin levels help exclude secondary polycythemia.

Symptoms

Facial and skin redness (plethora)
Itching (especially after a hot shower — aquagenic pruritus)
Headache, dizziness, and blurred vision
Splenomegaly (left upper quadrant fullness)
Fatigue and difficulty concentrating
Thrombosis symptoms (leg pain, shortness of breath, stroke)
Gout attacks (related to elevated uric acid)

Risk Factors

Carrying JAK2 V617F or exon 12 mutation
Age over 60
Family history of myeloproliferative neoplasm
Smoking (increases thrombosis risk)
Cardiovascular risk factors

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent facial redness, headache, and itching together
  • Detection of elevated hemoglobin, hematocrit, or platelets on complete blood count
  • After unexplained thrombosis
  • Persistent left upper quadrant fullness and fatigue

Treatment Methods

01
Phlebotomy: regular blood draws to maintain hematocrit <45 (men) / <42 (women)
02
Aspirin 100 mg/day: reduces thrombosis risk (if no high bleeding risk)
03
Hydroxyurea: cytoreductive therapy in high-risk cases (age >60 or thrombosis history)
04
Ruxolitinib (JAK1/2 inhibitor): in inadequate response or intolerance to hydroxyurea
05
Allopurinol for elevated uric acid
06
Anticoagulation if thrombosis develops

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.