Table 2

Criteria for the diagnosis of ET

WHO 2008BCSH 2010
Requires A1-A4 Requires A1-A3 or A1 + A3-A5 
    A1: Sustained platelet count >450 × 109/L     A1: Sustained platelet count >450 × 109/L 
    A2: Bone marrow showing increased numbers of enlarged, mature megakaryocytes; no significant increase of left-shift of granulopoiesis or erythropoiesis*     A2: Presence of an acquired pathogenetic mutation (eg, in JAK2 or MPL
    A3: Not meeting WHO criteria for PV, PMF, CML, MDS, or other myeloid neoplasm     A3: No other myeloid malignancy, especially PV, PMF, CML, or MDS 
    A4: Acquired mutation or clonal marker or no reactive cause for thrombocytosis     A4: No reactive cause for thrombocytosis and normal iron stores 
     A5: Bone marrow aspirate and trephine biopsy showing increased megakaryocyte numbers displaying a spectrum of morphology with predominant large megakaryocytes with hyperlobated nuclei and abundant cytoplasm. 
WHO 2008BCSH 2010
Requires A1-A4 Requires A1-A3 or A1 + A3-A5 
    A1: Sustained platelet count >450 × 109/L     A1: Sustained platelet count >450 × 109/L 
    A2: Bone marrow showing increased numbers of enlarged, mature megakaryocytes; no significant increase of left-shift of granulopoiesis or erythropoiesis*     A2: Presence of an acquired pathogenetic mutation (eg, in JAK2 or MPL
    A3: Not meeting WHO criteria for PV, PMF, CML, MDS, or other myeloid neoplasm     A3: No other myeloid malignancy, especially PV, PMF, CML, or MDS 
    A4: Acquired mutation or clonal marker or no reactive cause for thrombocytosis     A4: No reactive cause for thrombocytosis and normal iron stores 
     A5: Bone marrow aspirate and trephine biopsy showing increased megakaryocyte numbers displaying a spectrum of morphology with predominant large megakaryocytes with hyperlobated nuclei and abundant cytoplasm. 

BCSH indicates British Committee for Standards in Haematology; CML, chronic myeloid leukemia; ET, essential thrombocythemia; MDS, myelodysplastic syndromes; MPN, myeloproliferative neoplasm; PMF, primary myelofibrosis; PV, polycythemia vera; and WHO, World Health Organization.

*

Increased reticulin fibrosis excludes a diagnosis of ET

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