An elevated platelet count, typically referred to as thrombocytosis or thrombocythemia, is usually defined as an increase in platelet counts above 500,000/μl from a routine complete blood count measurement. However, some neoplastic hematologic disorders use a cutoff point of ≥450,000/μl to define thrombocytosis, as is the case with a provisional subtype of myelodysplastic/myeloproliferative overlap neoplasm (MDS/MPN) called refractory anemia with ring sideroblasts (RARS) associated with marked thrombocytosis (RARS-T) and essential thrombocythemia (ET) (Swerdlow et al., WHO classification of tumours of haematopoietic and lymphoid tissues, 2008). Occasionally platelet counts can increase to >1,000,000/μl and are referred to as extreme thrombocytosis. The etiologies of thrombocytosis are generally divided into primary or secondary causes. Primary causes are usually related to an underlying clonal hematologic cancer that causes autonomous proliferation of megakaryocytes. The most common diseases causing autonomous thrombocytosis are myeloproliferative neoplasms (MPN), myelodysplastic syndromes (MDS), and MDS/MPN overlap neoplasms. Secondary causes are typically reactive and account for the vast majority of all thrombocytosis cases encountered in clinical practice. Secondary thrombocytosis is primarily cytokine mediated and includes etiologies such as infection, connective tissue diseases, postsurgical, post-splenectomy, acute blood loss, iron-deficiency anemia, other cancers, and medications. Clinically, thrombocytosis, regardless of etiology, can also be associated with vasomotor (lightheadedness, headaches, atypical chest pain, erythromelalgia and visual disturbances, peripheral dysesthesias), thrombotic, and bleeding complications. However, the risk of bleeding, thrombotic complications, and vasomotor symptoms are usually seen with primary causes of thrombocytosis and only rarely in secondary thrombocytosis. Spurious causes of thrombocytosis can include mixed cryoglobulinemia and cytoplasmic fragments in patients with leukemia/lymphoma. We present here a series of clinical vignettes describing unique patient cases of thrombocytosis associated with various etiologies and their management.


Polycythemia Vera Essential Thrombocythemia Immune Thrombocytopenic Purpura International Prognostic Scoring System Polycythemia Vera Patient 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Nemours Center for Childhood Cancer ResearchA.I. duPont Hospital for ChildrenWilmingtonUSA
  2. 2.Taussig Cancer InstituteCleveland ClinicClevelandUSA

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