Polycythaemia Vera Symptoms: Early Signs & Warning Symptoms

Quick Glance: Common Polycythaemia Vera Symptoms

Here are the common symptoms and early warning signs that may occur with polycythaemia vera:

Persistent headaches
Dizziness or light-headedness
Tiredness and weakness
Itchy skin, particularly after a warm bath or shower
Blurred, double or disturbed vision
Shortness of breath
A flushed or reddened appearance, particularly on the face
Feeling of fullness or discomfort in the upper left abdomen

Symptoms of polycythaemia vera can vary between people. Some people have few or no symptoms, while others develop several symptoms as blood cell production increases or blood flow is affected.

Symptoms of Polycythaemia Vera That Should Not Be Ignored

The following table highlights symptoms that may indicate a blood clot, bleeding or another complication of polycythaemia vera and should be assessed promptly.

Symptom	What It May Indicate
Sudden weakness, numbness or difficulty speaking	Possible stroke or transient ischaemic attack caused by reduced blood flow to the brain
Sudden chest pain or pressure	Possible heart attack or another cardiovascular complication
Sudden difficulty breathing	Possible pulmonary embolism or another serious circulation problem
Pain, swelling or tenderness in one leg	Possible deep vein thrombosis (DVT)
Sudden or persistent loss of vision	Possible blockage of blood vessels supplying the eye
Unusual or persistent bleeding	Possible bleeding related to abnormal platelet function
Severe or persistent upper abdominal pain	May indicate a complication involving the spleen or abdominal blood vessels
Unexplained weight loss, night sweats or worsening fatigue	May require assessment for disease progression or another underlying condition

Polycythaemia vera is associated with an increased risk of both thrombosis and haemorrhage, so new or unexplained symptoms should not be ignored.

Why Do Polycythaemia Vera Symptoms Occur?

Polycythaemia vera develops when abnormal blood-forming cells in the bone marrow produce too many blood cells. The condition is usually associated with an acquired change in the JAK2 gene, which affects signalling involved in blood cell production. Red blood cell production increases, and white blood cells and platelet counts may also become elevated.

Increased Red Blood Cells and Blood Viscosity The excess red blood cells increase the proportion of blood occupied by cells, known as the haematocrit. This can make the blood more viscous and affect normal blood flow. Changes in blood flow may contribute to headaches, dizziness, fatigue and visual disturbances. Increased blood viscosity also contributes to the risk of blood clots in arteries or veins.
Itchy Skin After Bathing A characteristic symptom of polycythaemia vera is aquagenic pruritus, which is itching triggered by contact with water, particularly warm or hot water. The itching may occur without a visible skin rash and can affect large areas of the body.
Changes in Small Blood Vessels Changes in blood flow can affect small blood vessels and may cause burning, warmth, redness or pain in the hands and feet. This is known as erythromelalgia. Numbness or tingling may also occur.
Enlargement of the Spleen The spleen filters blood and helps remove older or abnormal blood cells. Increased blood cell production in polycythaemia vera can contribute to an enlarged spleen, known as splenomegaly. An enlarged spleen may cause discomfort or pain in the upper left abdomen and may make a person feel full after eating only a small amount.
Increased Platelet and White Blood Cell Production Although increased red blood cell production is the hallmark of polycythaemia vera, the bone marrow may also produce increased numbers of platelets and white blood cells. Abnormal platelet function can contribute to both clotting and bleeding problems.

Symptoms of Polycythaemia Vera That Require Immediate Medical Attention

The following symptoms of polycythaemia vera may indicate a serious blood clot, major bleeding or loss of blood flow and require urgent medical attention:

Sudden weakness or numbness, especially on one side of the body
Sudden difficulty speaking, understanding speech or maintaining balance
Sudden loss or major disturbance of vision
Severe or sudden chest pain or pressure
Sudden difficulty breathing or severe unexplained breathlessness
Coughing up blood
Sudden swelling and pain in one leg
Loss of consciousness
Severe, sudden abdominal pain

These symptoms can occur when a blood clot or another complication affects the brain, heart, lungs, limbs or other organs. Prompt medical attention is important because some complications of polycythaemia vera can be life-threatening.

Symptoms of Polycythaemia Vera-Specific Conditions

The table below shows common symptoms associated with complications or clinical manifestations of polycythaemia vera:

Complication	Symptoms That May Occur
Enlarged spleen (splenomegaly)	Discomfort or pain in the upper left abdomen and feeling full after eating small amounts
Erythromelalgia	Burning, warmth, redness and pain in the hands or feet
Gout	Sudden, painful swelling, often affecting the big toe
Blood clot	Symptoms depend on the affected blood vessel and may include chest pain, leg swelling, breathing difficulty or neurological symptoms
Bleeding	Nosebleeds, bleeding gums, easy bruising or gastrointestinal bleeding

Polycythaemia vera does not have clinically recognised symptom-based types that require a Type 1 versus Type 2 comparison. Its symptoms instead vary according to the effects of abnormal blood-cell production and complications such as thrombosis, bleeding or an enlarged spleen.

Can You Have Polycythaemia Vera Without Symptoms?

Yes. Polycythaemia vera can remain asymptomatic for years and may be discovered unexpectedly during a routine complete blood count (CBC) or another blood test that shows an abnormal blood count.

Early symptoms such as headaches, tiredness or dizziness can have many other causes and do not confirm polycythaemia vera. Persistent or unexplained symptoms should be assessed by a healthcare professional rather than self-diagnosed.

When Can Polycythaemia Vera Symptoms Become Life-Threatening?

Polycythaemia vera can become life-threatening when complications such as blood clots or severe bleeding affect vital organs. The table below explains the main serious complications and how they may present:

Life-Threatening Complication	What May Happen
Blood clots	Increased blood cell production and abnormal blood flow can increase the risk of arterial or venous thrombosis, which may lead to stroke, heart attack, DVT or pulmonary embolism.
Stroke	A blood clot can block blood flow to the brain, causing sudden weakness, numbness, speech difficulties, vision changes or loss of balance.
Heart attack	A blockage affecting blood flow to the heart can cause severe chest pain or pressure and breathlessness.
Pulmonary embolism	A blood clot can travel to the lungs and cause sudden breathlessness, chest pain, rapid breathing or coughing up blood.
Severe bleeding	Abnormal platelet function can contribute to significant bleeding, which requires urgent medical assessment.

Thrombosis and haemorrhage are recognised complications requiring particular attention in polycythaemia vera.

How is Polycythaemia Vera Diagnosed?

Symptoms alone cannot confirm polycythaemia vera. Doctors use the medical history, physical examination and laboratory investigations to determine whether polycythaemia vera is present.

The table below explains the main investigations that may be used when polycythaemia vera is suspected:

Diagnostic Test	Purpose
Complete blood count (CBC)	Measures red blood cells, white blood cells and platelets and can identify an abnormal blood count.
Haemoglobin and haematocrit	Help assess the concentration of red blood cells in the blood.
JAK2 genetic testing	Looks for JAK2 mutations, which are present in most people with polycythaemia vera.
Serum erythropoietin (EPO)	Measures the hormone that stimulates red blood cell production; levels are often low in polycythaemia vera.
Bone marrow aspiration or biopsy	Examines blood-forming cells and may form part of the diagnostic assessment when clinically indicated.
Physical examination	May identify findings such as an enlarged spleen and help guide further investigation.

Doctors may also perform other investigations to distinguish polycythaemia vera from secondary erythrocytosis, in which another condition causes an increase in red blood cell production. The diagnostic assessment uses a combination of findings rather than relying on symptoms or a single test alone.

Frequently Asked Questions (FAQs)

Q1. What is the first symptom of polycythaemia vera?

Ans: There is no single first symptom of polycythaemia vera. Headaches, tiredness, dizziness, itching after a warm shower and visual disturbances may occur early, while some people have no symptoms when polycythaemia vera is first detected.
Q2. Can polycythaemia vera develop without symptoms?

Ans: Yes. Polycythaemia vera can remain without noticeable symptoms for years.
Q3. Is itching a symptom of polycythaemia vera?

Ans: Yes. Itching, particularly after a warm bath or shower, is a recognised symptom of polycythaemia vera. This is known as aquagenic pruritus and may occur without an obvious skin rash.
Q4. Can polycythaemia vera cause headaches and dizziness?

Ans: Yes. Increased red blood cell production in polycythaemia vera can increase blood viscosity and affect blood flow, which may contribute to headaches, dizziness and light-headedness. These symptoms can also occur in many other conditions and do not confirm polycythaemia vera.
Q5. Which symptoms of polycythaemia vera should never be ignored?

Ans: Sudden chest pain, difficulty breathing, sudden weakness or numbness, speech difficulties, sudden vision loss and painful swelling of one leg are a few symptoms of polycythaemia vera that should not be ignored. These symptoms may indicate serious complications of polycythaemia vera, such as a heart attack, stroke, pulmonary embolism or DVT.
Q6. Are polycythaemia vera symptoms different in men and women?

Ans: The main symptoms of polycythaemia vera are generally similar in men and women.
Q7. When should I see a doctor for possible polycythaemia vera symptoms?

Ans: Speak to a healthcare professional if you have persistent or unexplained headaches, dizziness, tiredness, itching after bathing, visual changes or unusual bleeding, as they may indicate polycythaemia vera.
Q8. Can the intensity of polycythaemia vera symptoms vary?

Ans: Yes. Polycythaemia vera symptoms can range from mild or absent to troublesome and may change over time. Some people mainly experience fatigue or itching, while others develop symptoms related to an enlarged spleen, abnormal circulation, blood clots or bleeding.

References
- https://www.mayoclinic.org/diseases-conditions/polycythemia-vera/symptoms-causes/syc-20355850
- https://my.clevelandclinic.org/health/diseases/17742-polycythemia-vera
- https://bloodcancerunited.org/blood-cancer/myeloproliferative-neoplasms-mpns/polycythemia-vera-pv/sign-and-symptoms
- https://www.hopkinsmedicine.org/health/conditions-and-diseases/polycythemia-vera
- https://bloodcancer.org.uk/understanding-blood-cancer/polycythaemia-vera-pv/pv-symptoms/