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Polycythaemia Vera

Myeloproliferative neoplasm with bone-marrow overproduction of red cells.

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.

References (5)

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

What is Polycythaemia Vera?

Polycythaemia vera (PV) is a clonal myeloproliferative neoplasm in which the bone marrow overproduces red cells and, to a lesser degree, white cells and platelets. The JAK2 V617F mutation is found in over 95% of patients. Increased blood viscosity raises the risk of thrombosis and cardiovascular events.

The most feared complications of PV are arterial and venous thromboses: stroke, myocardial infarction, deep vein thrombosis and Budd-Chiari syndrome. In the long term the disease can progress to myelofibrosis or acute leukaemia.

The cornerstones of treatment are keeping the haematocrit below 45% (and below 42% in women) and reducing thrombotic risk. All patients receive phlebotomy and low-dose aspirin; high-risk patients also receive hydroxyurea or interferon-alpha.

Symptoms

Facial redness and burning
Itching, especially after bathing (aquagenic pruritus)
Headache and dizziness
Visual disturbance
Abdominal pain and fullness from splenomegaly
Fatigue and sweating
Symptoms of thrombosis: leg swelling, chest pain, stroke

Risk Factors

JAK2 V617F mutation
Family history of myeloproliferative neoplasm
Older age (median age at diagnosis ~60)
Previous thrombosis (high-risk category)
Cardiovascular risk factors (hypertension, diabetes, smoking)
High haematocrit and white-cell count
Splenomegaly

When to See a Doctor?

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

  • Elevated haemoglobin or haematocrit on a routine blood test
  • Itching, redness and headache occurring together
  • Symptoms of thrombosis (urgent attendance)
  • A palpable enlarged spleen
  • Patients with PV need regular haematology follow-up and phlebotomy

Treatment Methods

01
Phlebotomy: regular venesection to keep haematocrit at target
02
Low-dose aspirin: thrombosis prophylaxis in all patients
03
Hydroxyurea: cytoreduction in high-risk patients
04
Pegylated interferon-alpha: especially in younger patients and pregnant women
05
Ruxolitinib (JAK1/2 inhibitor): hydroxyurea-resistant or intolerant patients
06
Itch management: antihistamines, interferon, ruxolitinib

Which Department to Visit?

You can visit our Hematoloji 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.