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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Hematology

Symptoms and Warning Signs: What to Watch For

At a Glance

Essential Thrombocythemia (ET) can cause daily symptoms like fatigue, headaches, and a burning sensation in the hands or feet called erythromelalgia. Critically, ET increases the risk of severe blood clots and paradoxical bleeding, requiring immediate medical attention if red flag signs appear.

Living with Essential Thrombocythemia (ET) means becoming an expert on your own body. Because ET affects the “stickiness” and quantity of your platelets, symptoms can range from mild, daily nuisances to serious medical emergencies [1][2]. Understanding these signs helps you know when to mention a symptom at your next checkup and when to call for help immediately.

Common Daily Symptoms

Many people with ET experience “constitutional” or “microvascular” symptoms. While these are usually not life-threatening, they can significantly impact your quality of life:

  • Fatigue: A deep, persistent tiredness that doesn’t always improve with rest. To manage fatigue, try tracking your “energy envelope” to see when you have the most energy, prioritize gentle exercise like walking, and discuss any severe drops in energy with your doctor [3].
  • Headaches and Dizziness: Often caused by tiny blood clots in the small vessels of the brain, affecting blood flow [1][2].
  • Visual Disturbances: You may experience blurred vision, “spots,” or temporary flashes of light [1].
  • Erythromelalgia: This is a hallmark symptom of ET. It causes a painful, burning sensation, usually in the hands or feet, accompanied by redness (erythema), warmth, and swelling [4][2]. It is caused by platelets temporarily blocking the smallest blood vessels [2].

The Paradox: When High Platelets Cause Bleeding

It seems backward, but having too many platelets can sometimes make it harder for your blood to clot. This phenomenon is known as Acquired von Willebrand Syndrome (AVWS) [5][6].

When platelet counts become extremely high (often over 1 million), they can “soak up” and destroy a specific protein in your blood called von Willebrand factor, which is absolutely necessary for normal clotting [7][8]. This can lead to:

  • Frequent or heavy nosebleeds [9].
  • Bleeding gums [9].
  • Easy bruising or heavy menstrual periods [10].
  • Prolonged bleeding from minor cuts [6].

CRITICAL WARNING: Because of this bleeding risk, if your platelet count is extremely high, never start taking over-the-counter aspirin without your doctor’s explicit approval. Taking aspirin while you have AVWS can trigger severe, potentially life-threatening hemorrhage [7][6].

Critical Warning Signs

Because the primary risk of ET is the formation of a major blood clot (thrombosis), it is vital to recognize the “red flags” that require emergency medical attention [11][7].

Clarifying the Boundary: Erythromelalgia vs. Severe Ischemia

While erythromelalgia causes uncomfortable redness and burning, severe ischemia is an emergency where the tissue is starved of oxygen. If the pain in a toe or finger becomes suddenly intense, turns cold, and the area turns blue, purple, or black, go to the emergency room immediately [4].

Seek Emergency Care Immediately If You Experience:

Concern Symptoms to Watch For
Stroke Sudden weakness or numbness (especially on one side), facial drooping, difficulty speaking, or sudden confusion [12][13].
Heart Attack Chest pain, pressure, or discomfort; shortness of breath; or pain radiating to the jaw, neck, or arms [14][15].
Pulmonary Embolism Sudden shortness of breath, sharp chest pain when breathing in, or a rapid heart rate.
Deep Vein Thrombosis Swelling, pain, warmth, or redness in one leg or arm [16].
Splenic Infarction Sudden, severe pain in the upper left side of your abdomen, sometimes radiating to the left shoulder [14][15].
Severe Ischemia A toe or finger that becomes intensely painful, cold, and turns blue, purple, or black [4][17].

If you experience any of these signs, call 911 or your local emergency services immediately. Inform the emergency responders and hospital staff that you have Essential Thrombocythemia (ET). [13]

Common questions in this guide

Why do high platelets cause bleeding in Essential Thrombocythemia?
Extremely high platelet counts can destroy a specific protein in your blood needed for normal clotting. This leads to a condition called Acquired von Willebrand Syndrome (AVWS), which paradoxically makes it harder for your blood to clot and can cause easy bruising or nosebleeds.
What is erythromelalgia and why does it happen?
Erythromelalgia is a hallmark symptom of ET caused by platelets temporarily blocking the smallest blood vessels. It causes a painful, burning sensation, typically in the hands or feet, accompanied by redness, warmth, and swelling.
Can I take aspirin for my headaches if I have ET?
You should never start taking over-the-counter aspirin without your doctor's explicit approval. If your platelet count is extremely high, you may have an increased bleeding risk, and taking aspirin can trigger a severe, potentially life-threatening hemorrhage.
When should I go to the emergency room for ET symptoms?
Seek immediate medical attention if you experience signs of a major blood clot, such as sudden weakness or numbness on one side, severe chest pain, shortness of breath, or if a toe or finger becomes intensely painful, cold, and turns blue or black.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Am I at a higher risk for clotting or bleeding right now based on my current platelet count and mutation status?
  2. 2.Should I be screened for Acquired von Willebrand Syndrome (AVWS) before any upcoming surgeries or dental work?
  3. 3.Is my current medication dose sufficient to manage my headaches and other microvascular symptoms?
  4. 4.If I experience an erythromelalgia flare-up, what is the best way to manage the pain at home?
  5. 5.Are there specific warning signs for a 'splenic infarction' that I should be on the lookout for?
  6. 6.What are some specific strategies you recommend for managing ET-related fatigue?

Questions For You

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References

References (17)
  1. 1

    Essential Thrombocythemia: The Dermatologic Point of View.

    Cozzani E, Iurlo A, Merlo G, et al.

    Clinical lymphoma, myeloma & leukemia 2015; (15(12)):739-47.

    PMID: 26432058
  2. 2

    A toe ulcer in a patient with essential thrombocythemia successfully treated with a combination of anagrelide and prednisolone.

    Sugai T, Yamaguchi Y, Narahira A, et al.

    Dermatologic therapy 2022; (35(2)):e15221 doi:10.1111/dth.15221.

    PMID: 34846771
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    Marked elevation of serum lactate dehydrogenase in primary myelofibrosis: clinical and prognostic correlates.

    Shah S, Mudireddy M, Hanson CA, et al.

    Blood cancer journal 2017; (7(12)):657 doi:10.1038/s41408-017-0024-9.

    PMID: 29249804
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    Toe Gangrene as the First Presenting Symptom of Essential Thrombocythemia: A Case Report.

    Altayyan MM, Abu-Tineh M, Alshurafa A, et al.

    Cureus 2023; (15(7)):e42388 doi:10.7759/cureus.42388.

    PMID: 37621813
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    Acquired von Willebrand Syndrome in a 17-Year-Old With Essential Thrombocythemia: A Case Report With Literature Review.

    Youn L, Kuta A, Srinivasan M, et al.

    Cureus 2024; (16(3)):e55668 doi:10.7759/cureus.55668.

    PMID: 38586809
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    Case report: Peri-procedural hydroxyurea helps minimize bleeding in patients with Essential Thrombocythemia associated with acquired von Willebrand syndrome.

    Kogan L, Price R, Kotchetkov R

    Frontiers in oncology 2024; (14()):1326209 doi:10.3389/fonc.2024.1326209.

    PMID: 38361779
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    Increased cleavage of von Willebrand factor by ADAMTS13 may contribute strongly to acquired von Willebrand syndrome development in patients with essential thrombocythemia.

    Kubo M, Sakai K, Hayakawa M, et al.

    Journal of thrombosis and haemostasis : JTH 2022; (20(7)):1589-1598 doi:10.1111/jth.15717.

    PMID: 35352474
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    Increased von Willebrand factor levels in polycythemia vera and phenotypic differences with essential thrombocythemia.

    Sacco M, Ranalli P, Lancellotti S, et al.

    Research and practice in thrombosis and haemostasis 2020; (4(3)):413-421 doi:10.1002/rth2.12315.

    PMID: 32211575
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    Same Syndrome, Different Causes and Treatment: Path to Diagnosis and Management of Two Interesting Cases of Acquired von Willebrand Syndrome.

    Monti M, Massari E, Rosetti M, et al.

    Seminars in thrombosis and hemostasis 2024; (50(2)):307-309 doi:10.1055/s-0043-1772837.

    PMID: 37640045
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    Intravenous Immunoglobulin offers temporary improvement in acquired von Willebrand syndrome due to monoclonal gammopathy: A case report.

    Zablonski KG, Rajkumar A, Nayak L

    EJHaem 2024; (5(4)):833-837 doi:10.1002/jha2.969.

    PMID: 39157619
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    Aquagenic pruritus in essential thrombocythemia is associated with a higher risk of thrombosis.

    Le Gall-Ianotto C, Le Calloch R, Couturier MA, et al.

    Journal of thrombosis and haemostasis : JTH 2019; (17(11)):1950-1955 doi:10.1111/jth.14588.

    PMID: 31344312
  12. 12

    The comorbidity of acute ischemic stroke and splenic infarction resulting from essential thrombocythemia.

    Yuan J, Wu Y, Hao J, Hu W

    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2018; (39(10)):1787-1790 doi:10.1007/s10072-018-3462-7.

    PMID: 29984396
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    Recurrent Stroke with Rapid Development of Intracranial Artery Stenosis and Subsequent Successful Mechanical Thrombectomy in Essential Thrombocythemia.

    Kato Y, Iijima S, Kohyama S, et al.

    Internal medicine (Tokyo, Japan) 2024; (63(6)):847-852 doi:10.2169/internalmedicine.2189-23.

    PMID: 37495531
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    Splenic Infarct as the Presenting Manifestation of Essential Thrombocythemia.

    Khade S, Khera S, Varshney VK, et al.

    Ochsner journal 2022; (22(2)):188-191 doi:10.31486/toj.21.0073.

    PMID: 35756582
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    Atypical Myocardial Infarction with Apical Thrombus and Systemic Embolism: A Rare Presentation of Likely JAK2 V617F-Positive Myeloproliferative Neoplasm.

    Atere M, Al-Zakhari R, Collins J, et al.

    Case reports in oncological medicine 2020; (2020()):9654048 doi:10.1155/2020/9654048.

    PMID: 32685224
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    Incidence of Thrombosis at Different Sites During the Follow-Up Period in Essential Thrombocythemia: A Systematic Review and Meta-Analysis.

    Wang D, Yu X, Sun Y, et al.

    Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2023; (29()):10760296231181117 doi:10.1177/10760296231181117.

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    Gangrene and osteomyelitis of the toe in a previously healthy patient due to undiagnosed essential thrombocythemia.

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This page is for informational purposes only and does not replace professional medical advice. Always consult your hematologist if you experience new or worsening symptoms of Essential Thrombocythemia, and seek immediate emergency care for signs of major blood clots.

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