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==Overview==
==Overview==
==Diagnostic criteria==
The diagnosis of essential thrombocytosis requires the presence of a persistent thrombocytosis of greater than 600 x10<sup>9</sup>/L in the absence of an alternative cause.
The following revised diagnostic criteria for essential thrombocytosis were proposed in 2005 <ref name=Campbell>Campbell PJ, Green AR. ''Management of Polycythemia Vera and Essential Thrombocythemia.'' Hematology (Am Soc Hematol Educ Program). 2005;:201-8. PMID 16304381</ref>. The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6.
* A1. Platelet count > 600 x 10<sup>9</sup>/L for at least 2 months
* A2. Acquired V617F JAK2 mutation present
* B1. No cause for a reactive thrombocytosis
** normal inflammatory indices
* B2. No evidence of iron deficiency
** stainable iron in the bone marrow or normal red cell mean corpuscular volume
* B3. No evidence of [[polycythemia vera]]
** hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores
* B4. No evidence of chronic myeloid leukemia
But the Philadelphia chromosome may be present in up to 10% of cases. Patients withe the Philadelphia chromosome have a potential for the development of acute leukemia, especially acute lymphocytic leukemia.
* B5. No evidence of [[myelofibrosis]]
** no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0–4 scale)
* B6. No evidence of a myelodysplastic syndrome
** no significant dysplasia
** no cytogenetic abnormalities suggestive of myelodysplasia


==References==
==References==

Revision as of 22:02, 20 January 2012

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Diagnostic criteria

The diagnosis of essential thrombocytosis requires the presence of a persistent thrombocytosis of greater than 600 x109/L in the absence of an alternative cause.

The following revised diagnostic criteria for essential thrombocytosis were proposed in 2005 [1]. The diagnosis requires the presence of both A criteria together with B3 to B6, or of criterion A1 together with B1 to B6.

  • A1. Platelet count > 600 x 109/L for at least 2 months
  • A2. Acquired V617F JAK2 mutation present
  • B1. No cause for a reactive thrombocytosis
    • normal inflammatory indices
  • B2. No evidence of iron deficiency
    • stainable iron in the bone marrow or normal red cell mean corpuscular volume
  • B3. No evidence of polycythemia vera
    • hematocrit < midpoint of normal range or normal red cell mass in presence of normal iron stores
  • B4. No evidence of chronic myeloid leukemia

But the Philadelphia chromosome may be present in up to 10% of cases. Patients withe the Philadelphia chromosome have a potential for the development of acute leukemia, especially acute lymphocytic leukemia.

  • B5. No evidence of myelofibrosis
    • no collagen fibrosis and ≤ grade 2 reticulin fibrosis (using 0–4 scale)
  • B6. No evidence of a myelodysplastic syndrome
    • no significant dysplasia
    • no cytogenetic abnormalities suggestive of myelodysplasia

References

  1. Campbell PJ, Green AR. Management of Polycythemia Vera and Essential Thrombocythemia. Hematology (Am Soc Hematol Educ Program). 2005;:201-8. PMID 16304381


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