What are the diagnostic criteria for essential thrombocytosis?

Updated: Dec 24, 2018
  • Author: Koyamangalath Krishnan, MD, FRCP, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Essential thrombocythemia is a diagnosis of exclusion that is based on the following numeric criteria (adapted from Hoffman R. Primary thrombocythemia. In: Hoffman R, Benz EJ Jr, Shattil SJ, et al, eds. Hematology: Basic Principles and Practice. 3rd ed. Philadelphia, Pa: Churchill Livingstone; 2000:1188-204). [4] Patients who meet criteria 1-5 and more than three of criteria 6-11 are considered to have essential thrombocytosis.

  1. Platelet count greater than 600,000/mm3 on two occasions, separated by a 1-month interval

  2. Absence of an identifiable cause of secondary thrombocytosis

  3. Normal red blood cell mass

  4. Absence of significant bone marrow fibrosis (ie, less than one third of the bone marrow)

  5. Absence of the Philadelphia chromosome (Ph) by karyotyping or absence of the bcr-abl fusion product

  6. Splenomegaly by physical examination or ultrasonography

  7. Bone marrow hypercellularity with megakaryocyte hyperplasia

  8. Presence of abnormal bone marrow hematopoietic progenitor cells as determined by the growth of endogenous erythroid cells and/or megakaryocyte colonies with increased sensitivity to interleukin-3 (IL-3)

  9. Normal levels of CRP and IL-6

  10. Absence of iron deficiency anemia, as documented by either a normal bone marrow–stainable iron or normal serum ferritin level

  11. In females, demonstration of clonal hematopoiesis by restriction fragment length polymorphism (RFLP) analysis of genes present on the X chromosome

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