Essential Thrombocythemia

Essential Thrombocythemia

A person with essential thrombocythemia (ET) has an excess amount of platelets in the blood due to a disorder of the bone marrow.  A change in the bone marrow stem cell DNA causes the overproduction of platelet-forming cells in the bone marrow.  What causes essential thrombocythemia to occur is not yet known.  ET is one of the myeloproliferative disorders (MPD).

There are an estimated 0.1 to 2.4 new cases of essential thrombocythemia per 100,000 people per year.  ET may occur at any age but tends to be more common in adults.  It affects both women and men of all ethnic backgrounds.

Symptoms

Many people with essential thrombocythemia have no symptoms.  Others may have symptoms such as:

  • skin problems such as redness, burning, or itching
  • pain in the feet and hands
  • a mildly enlarged spleen
  • headache, dizziness, weight loss
  • abnormal bleeding (rarely)
  • pregnancy complications
  • abnormal blood clotting (most common problem)

If a blood clot forms it may block blood flow to a part of the body.  This may cause serious problems such as stroke (a blood clot in the brain) or heart attack (a blood clot in the heart). People who smoke or drink alcohol increase their risk of developing a blood clot.

Many people with essential thrombocythemia have no signs or symptoms.  The first indication you have to the disorder may be the development of a blood clot (thrombus).  Although clots can develop anywhere in your body, with ET, they occur most often in your brain, hands and feet.

Signs and symptoms depend on where the clot forms.  They include:

  1. Headache
  2. Dizziness or lightheadedness
  3. Chest pain
  4. Weakness
  5. Fainting
  6. Temporary vision changes
  7. Numbness or tingling of the hands and feet
  8. Redness, throbbing and burning pain in the hands and feet (erythromelalgia)
  9. Mildly enlarged spleen

Less commonly, ET may cause bleeding, especially if your platelet count is extremely high (more than 1 million platelets per microliter of blood).  Bleeding may take the form of:

  1. Nosebleeds
  2. Bruising
  3. Bleeding from your mouth or gums
  4. Bloody stool

A blood clot may cause a transient ischemic attack (TIA) a temporary interruption of blood flow to part of the brain or stroke.  Signs and symptoms develop suddenly and include:

  1. Weakness or numbness of your face, arm or leg, usually on one side of your body
  2. Difficulty speaking or understanding speech (aphasia)
  3. Blurred, double or decreased vision

When to see a doctor

If you have any signs or symptoms of abnormal blood clotting or bleeding, see your doctor.

If you develop signs or symptoms of a TIA or stroke, such as numbness or paralysis on one side of your body, seek medical attention immediately.

The pathologic basis for this disease is unknown.  However, essential thrombocythemia resembles polycythemia vera in that cells of the megakaryocytic series are more sensitive to growth factors. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis.  A mutation in the JAK2 kinase (V617F) has been found  to be associated with essential thrombocythemia in around forty to fifty percent of cases.  About three to four percent of such cases go on to develop acute leukemia.  JAK2 is a member of the Janus kinase family.  This mutation may be helpful in making a diagnosis or as a target for future therapy.

Lifestyle and home remedies

Take extra care to reduce your risk of developing blood clots if you have essential thrombocythemia.  Healthy lifestyle habits can lower your risk of developing conditions that may contribute to blood clotting.  These conditions include diabetes, high blood pressure and high blood cholesterol.  Take steps to:

  • Eat healthy foods.  Choose a varied diet rich in whole grains, vegetables and fruits and low in saturated fats.  Try to avoid trans fats.   Portion control to maintain a normal weight.
  • Increase your physical activity.  Aim for at least 30 minutes of moderate physical activity a day.  Take a brisk daily walk.  Ride your bike.  Swim laps.  If you can not fit in a long workout, break it up into smaller sessions spread throughout the day
  • Achieve or maintain normal weight.  Being overweight or obese increases the pressure in the veins in your pelvis and legs and is a risk factor for conditions such as high blood pressure, which increases your risk of blood clotting.
  • Stop smoking.  Smoking affects blood clotting and circulation.

If your ET increases your tendency to bleed, take extra precautions to keep from injuring yourself.  Follow these suggestions:

  1. Avoid playing contact sports or engaging in other activities that could be dangerous or could cause you to fall.
  2. Use a softer toothbrush and waxed floss.
  3. Avoid shaving cuts.  Shave with an electric razor.
  4. Be cautious with household tasks involving knives, scissors and other sharp tools.

If an individual has no symptoms, essential thrombocythemia may be diagnosed from a blood test (platelet count) during a routine physical examination.  Upon examination,  an enlarged spleen may be observed.

Treatment

Treatment depends on the level of platelets in the blood and the risk of clotting or bleeding complications.  Plateletpheresis (removing platelets from the blood) may be used as an emergency treatment to quickly reduce an extremely high platelet level.  Longer-term treatment may be observation of the individual's status through regular doctor visits  (if the risk for blood clots is low).  Those with higher risk may be treated with medications such as hydroxyurea, anagrelide, or interferon alpha.
Individuals with essential thrombocythemia usually have a normal lifespan with proper medical treatment. 
However,  if a stroke, heart attack, or other serious complication occurs, the individual may become permanently disabled.

Prognosis

Patients with essential thrombocytosis have near-normal life expectancy because of the low rate of leukemic conversion.  The major morbidity factor is the increased risk of hemorrhagic complications.  Essential thrombocytosis is a slowly progressive disorder with long asymptomatic periods punctuated by thrombotic or hemorrhagic events.  The average survival of patients with this disorder is 12 to 15 years, and may be much longer in younger patients.
November, 2011
New drug given approval by FDA:   Jakafi  ™ (ruxolitinib).  Treatment:  polycythemia vera and essential thrombocythemia (ET)

Medical Marijuana
Use organically grown medical marijuana.  An indica x hybrid—made into a (whole plant extract) tincture.
Taken under the tongue (1-2 drops) twice a day.  Medical marijuana:

  • anti-inflammatory properties
  • cannabinoids stop the growth of certain cancer cells
  • anti-stress properties
  • anti-microbial properties
  • relieves hypertension
  • relieves burning hands and feet
  • relieves numbness and tingling

References


Mesa R, Silverstein M, Jacobsen S, Wollan P, Tefferi A (1999). "Population-based incidence and survival figures in essential thrombocythemia and agnogenic myeloid metaplasia: an Olmsted County Study, 1976-1995". Am J Hematol 61 (1): 10–5. doi:10.1002/(SICI)1096-8652(199905)61:1<10::AID-AJH3>3.0.CO;2-I. PMID 10331505.
Kutti J, Ridell B (2001). "Epidemiology of the myeloproliferative disorders: essential thrombocythaemia, polycythaemia vera and idiopathic myelofibrosis". Pathol Biol (Paris) 49 (2): 164–6. PMID 11317963.
Hoffman: Hematology: Basic Principles and Practice, 4th ed., 2005 Churchill Livingstone, Chapter 71.
Kralovics R, Passamonti F, Buser AS, Teo SS, et al. (2005). "A gain-of-function mutation of JAK2 in myeloproliferative disorders". N Engl J Med 352 (17): 1779–90. doi:10.1056/NEJMoa051113. PMID 15858187.
Baxter EJ, Scott LM, Campbell PJ, et al. (2005). "Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders". Lancet 365 (9464): 1054–61. doi:10.1016/S0140-6736(05)71142-9. PMID 15781101. http://linkinghub.elsevier.com/retrieve/pii/S0140-6736(05)71142-9.
Levine RL, Wadleigh M, Cools J, et al. (2005). "Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis". Cancer Cell 7 (4): 387–97. doi:10.1016/j.ccr.2005.03.023. PMID 15837627. http://linkinghub.elsevier.com/retrieve/pii/S1535-6108(05)00094-2.
Campbell PJ, Green AR (2005). "Management of polycythemia vera and essential thrombocythemia". Hematology Am Soc Hematol Educ Program 2005: 201–8. doi:10.1182/asheducation-2005.1.201. PMID 16304381. http://www.asheducationbook.org/cgi/pmidlookup?view=long&pmid=16304381.
Harrison CN, Campbell PJ, Buck G, et al. (2005). "Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia". N. Engl. J. Med. 353 (1): 33–45. doi:10.1056/NEJMoa043800. PMID 16000354. http://content.nejm.org/cgi/pmidlookup?view=short&pmid=16000354&promo=ONFLNS19.


The Leukemia & Lymphoma Society. Essential or Primary Thrombocythemia
Arizona Telemedicine Program. Myeloproliferative Disorders


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