Essential Thrombocytosis Treatment & Management

  • Author: Asheesh Lal, MBBS, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 10, 2012
 

Medical Care

  • Minimizing risk factors: Individualize the treatment in patients with essential thrombocytosis (primary thrombocythemia) based on the risk factors for thrombohemorrhagic complications. Risk factors include the following:
    • Age 60 years or older
    • History of thrombosis
    • Platelet count greater than 1.5 million x 109/L, which is paradoxically associated with an increased risk of gastrointestinal tract bleeding in young women
    • Obesity
    • Cardiovascular risk factors such as smoking, hypertension, and hypercholesterolemia
    • Markers of hypercoagulability such as factor V Leiden and antiphospholipid antibodies[3]
  • Medications
    • Consider administering hydroxyurea,[5, 23] anagrelide,[23] interferon alfa, or phosphorous-32 (32 P) (common cytoreductive drugs that decrease the platelet count).
    • In addition, suggest low-dose aspirin, which may be useful in treating patients with symptoms of microvascular occlusion (eg, erythromelalgia).
    • In emergencies, plateletpheresis may be useful to achieve a rapid decrease in platelet counts in the setting of acute thrombosis and/or marked thrombocytosis.
    • Observation may be appropriate for low-risk patients (ie, those lacking any of the previously mentioned risk factors). Low-risk patients experience lower rates of thrombosis or bleeding. Generally, significantly increased thrombohemorrhagic risk is not associated with surgery or pregnancy in low-risk patients.
  • Treatment choices
    • Recommend lifestyle modifications (eg, weight loss for obese patients, smoking cessation for smokers) to all patients with essential thrombocytosis (primary thrombocythemia).
    • Provide cytoreductive therapy to best treat high-risk patients (eg, those agedover 60 y, those with history of thrombosis or platelets greater than 1.5 million/µL). A randomized study comparing hydroxyurea versus observation in patients at high risk for thrombosis showed a marked decrease in the number of thrombotic episodes in the hydroxyurea arm.[5]
    • Another randomized study in which high-risk patients (previous thrombosis, age >60 y or platelets >1000 x 109/L) were randomized to receive hydroxyurea plus aspirin or anagrelide plus aspirin.[23] Compared with hydroxyurea plus aspirin, treatment with anagrelide plus aspirin was associated with increased rates of arterial thrombosis, major hemorrhage, and myelofibrotic transformation, but a decreased rate of venous thromboembolism.[23]
    • In view of these studies, hydroxyurea should be considered as cytoreductive agent of choice for most high-risk patients with essential thrombocytosis (primary thrombocythemia). Further choice of the cytoreductive agent should also be based on patient factors (eg, age, child-bearing potential, cost, life expectancy, comorbidities). A combination of cytoreductive agents may be needed in cases difficult to manage by single-agent therapy.
    • Anagrelide was associated with a higher incidence of development of myelofibrosis as opposed to hydroxyurea. Whether this represents an effect of anagrelide with increased production of profibrotic cytokines versus the natural course of essential thrombocytosis (primary thrombocythemia) remains unclear.
    • Low-dose aspirin with cytoreductive therapy or observation may help treat intermediate-risk patients (eg, those who do not fit into either high-risk or low-risk groups. Recommend lifestyle modifications (eg, weight loss for obese patients, smoking cessation for smokers).
    • Low-risk patients may be managed with low-dose aspirin alone or with observation.
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Surgical Care

  • Patients with essential thrombocytosis (primary thrombocythemia) undergoing surgery are at increased risk for bleeding and thrombosis.
  • Administer cytoreductive therapy to decrease the platelet count to the reference range before surgery.
  • Avoid splenectomy because it can markedly increase the platelet count and the risk of both hemorrhagic and thrombotic events.
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Consultations

A hematologist can help manage patients with essential thrombocytosis (primary thrombocythemia) as well as monitor the cytoreductive therapy.

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Contributor Information and Disclosures
Author

Asheesh Lal, MBBS, MD  Physician, Department of Internal Medicine, Lexington Medical Center

Asheesh Lal, MBBS, MD is a member of the following medical societies: American Society of Clinical Oncology and American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Wadie F Bahou, MD  Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Marcel E Conrad, MD  Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

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Peripheral blood smear in essential thrombocytosis showing increased platelet numbers. Courtesy Wei Wang, MD, and John Lazarchick, MD; Department of Pathology, Medical University of South Carolina.
Bone marrow biopsy in essential thrombocytosis showing increased megakaryocytes. Courtesy Wei Wang, MD, and John Lazarchick, MD; Department of Pathology, Medical University of South Carolina.
 
 
 
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