Essential thrombocytosis diagnostic criteria
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]
Overview
The diagnosis of essential thrombocytosis is made when all four of the diagnostic criteria are met which include: sustained elevation of platelet counts > 450,000,000 × 10³/L, bone marrow biopsy specimen showing proliferation, mainly of the megakaryocytic lineage with increased numbers of enlarged, mature megakaryocytes and no significant increase or left-shift of neutrophil granulopoiesis or erythropoiesis, not meeting WHO criteria for polycythemia vera (PV), PMF, CML, MDS, or other myeloid neoplasm, and demonstration of JAK2 617VF or other clonal marker, or in the absence of a clonal marker, no evidence for reactive thrombocytosis.[1]
Diagnostic criteria
- Diagnosis requires meeting all the following four criteria as proposed by WHO in their revised WHO criteria for essential thrombocythemia[1] :
- 1. Sustained platelet count > 450,000,000 × 10³/L*
- 2. Bone marrow biopsy specimen showing proliferation mainly of the megakaryocytic lineage with increased numbers of enlarged, mature megakaryocytes; no significant increase or left-shift of neutrophil granulopoiesis or erythropoiesis
- 3. Not meeting WHO criteria for polycythemia vera,† PMF,‡ CML,§ MDS,¶ or other myeloid neoplasm
- 4. Demonstration of JAK2617VF or other clonal marker, or in the absence of a clonal marker, no evidence for reactive thrombocytosis
* During the work-up period. † Requires the failure of iron replacement therapy to increase hemoglobin level to the polycythemia vera (PV) range in the presence of decreased serum ferritin. Exclusion of PV is based on hemoglobin and hematocrit levels, and red cell mass measurement is not required. ‡ Requires the absence of relevant reticulin fibrosis, collagen fibrosis, peripheral blood leukoerythroblastosis, or markedly hypercellular marrow for age accompanied by megakaryocyte morphology that is typical for primary myelofibrosis small to large with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous or irregularly folded nuclei and dense clustering. § Requires the absence of BCR-ABL. ¶ Requires absence of dyserythropoiesis and dysgranulopoiesis. Causes of reactive thrombocytosis include iron deficiency, splenectomy, surgery, infection, inflammation, connective tissue disease, metastatic cancer, and lymphoproliferative disorders. However, the presence of a condition associated with reactive thrombocytosis does not exclude the possibility of essential thrombocytosis if the first three criteria are met.
References
- ↑ 1.0 1.1 Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID http://dx.doi.org/10.1182/blood-2007-04-083501 Check
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