PV may be associated with various clinical manifestations. Individual patients may experience some, none, or many of these manifestations [5][6][7]:

Neurologic or microvascular manifestations may include:

• Headaches

• Dizziness

• Visual disturbances

• Tinnitus

• Erythromelalgia (burning sensation in extremities)

Constitutional symptoms may include:

• Fatigue

• Night sweats

• Unintentional weight loss

• Low-grade fever

Other manifestations may include:

• Pruritus (itching), which may be triggered or worsened by warm water exposure

• Splenomegaly (enlarged spleen), which may cause abdominal discomfort or early satiety

• Thromboembolic events

• Bleeding manifestations in some patients

Important: The presence or absence of specific symptoms does not confirm or exclude a diagnosis of PV. Many of these manifestations are nonspecific and may occur in other conditions. Diagnostic evaluation by a healthcare professional is essential.

Some published studies have examined whether clinical presentations and outcomes in PV differ by sex. Key observations from the literature include [1][2][3][4]:

Diagnostic considerations:

Some reports suggest that diagnosis may be delayed in female patients in certain clinical contexts. Factors that may contribute to this observation include historical differences in reference ranges for hemoglobin/hematocrit values and the potential masking effect of iron deficiency [1][9].

Symptom burden:

Some patient-reported outcome studies have suggested differences in symptom burden, particularly regarding fatigue and quality of life measures. However, symptom experience is highly individual and influenced by multiple factors beyond sex alone [1][2].

Thromboembolic patterns:

Published data have suggested potential differences in the types of thromboembolic events, with some studies reporting relatively higher rates of venous thrombosis in female patients and arterial thrombosis in male patients. Clinical significance of these observations continues to be investigated [1][8].

Important: These observations are based on population-level studies and may not apply to individual patients. Risk assessment and management decisions must be individualized based on comprehensive clinical evaluation.

Reproductive considerations:

Pregnancy in patients with PV requires specialized management due to potential risks including pregnancy loss, fetal growth restriction, and maternal thromboembolic complications. Management should be coordinated between hematology and maternal-fetal medicine specialists. Treatment selection during pregnancy requires careful consideration of both maternal and fetal safety [3].

Iron status:

Iron deficiency may occur in PV patients for various reasons including phlebotomy therapy or menstrual blood loss. Iron deficiency may affect red blood cell indices and potentially complicate diagnostic evaluation [2].

Bleeding considerations:

Extreme thrombocytosis in some PV patients may be associated with acquired von Willebrand syndrome, which can increase bleeding risk. This requires appropriate monitoring and management [8].

Critical: Patients with specific concerns including pregnancy planning, pregnancy, or unusual bleeding should discuss these with their healthcare provider promptly. These situations may require specialized evaluation and management adjustments

Diagnosis of PV requires comprehensive evaluation including [2][4]:

• Complete blood count with differential

• JAK2 mutation testing

• Bone marrow examination in selected cases

• Exclusion of secondary causes of erythrocytosis

• Assessment of iron status

Diagnostic criteria have been established by expert groups and should be applied by qualified hematologists [2][4].

Management of PV is individualized based on multiple factors including risk stratification, symptom burden, comorbidities, and patient-specific circumstances [2][4].

Management approaches may include:

• Phlebotomy to maintain target hematocrit levels

• Low-dose aspirin for cardiovascular risk reduction in appropriate patients

• Cytoreductive therapy in select patients based on risk assessment

• Symptom management strategies

• Regular monitoring for complications

Treatment selection should consider multiple factors including age, comorbidities, pregnancy plans, and individual patient circumstances [2][4].

PV requires ongoing management by or in consultation with a hematologist experienced in myeloproliferative neoplasms. Key aspects of care include:

• Accurate diagnosis and risk stratification

• Individualized treatment planning

• Regular monitoring and assessment

• Management of complications

• Coordination with other specialists as needed

• What symptoms should I monitor for?

• How is my individual risk assessed?

• What treatment approach is recommended for my specific situation?

• What monitoring is required in my case?

• What symptoms or changes should prompt me to contact you?

• If I am considering pregnancy, what specialized care is needed?

• What are the potential complications I should be aware of?

• How can I best manage my symptoms?

1. Palandri F, Mora B, Gangat N, Catani L. Is there a gender effect in polycythemia vera? Ann Hematol. 2021;100(1):11-25.

2. Tremblay D, Kremyanskaya M, Mascarenhas J, Hoffman R. Diagnosis and treatment of polycythemia vera: a review. JAMA. 2025;333(2):153-160.

3. Tefferi A, Vannucchi AM, Barbui T. Polycythemia vera: historical oversights, diagnostic details, and therapeutic views. Leukemia. 2021;35(12):3339-3351.

4. Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk stratification, and management. Am J Hematol. 2023;98(9):1465-1487.

5. Spivak JL. Polycythemia vera. Curr Treat Options Oncol. 2018;19(2):12.

6. Stuart BJ, Viera AJ. Polycythemia vera. Am Fam Physician. 2004;69(9):2139-2144.

7. Spivak JL. Polycythemia vera: myths, mechanisms, and management. Blood. 2002;100(13):4272-4290.

8. Landolfi R, Nicolazzi MA, Porfidia A, Di Gennaro L. Polycythemia vera. Intern Emerg Med. 2010;5(5):375-384.

9. Calabresi P, Meyer OO. Polycythemia vera. I. Clinical and laboratory manifestations. Ann Intern Med. 1959;50(5):1182-1202.