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Volume 1 | Issue 1 Abstract The WHO defined JAK2V617F mutated myeloproliferative neoplasms (MPN) consist of normocellular essential thrombocythemia (ET), ET with features of early PV (prodromal PV), ET with hypercelular megakaryocytic granulocytic myeloproliferation (ET.MGM), and various stages of polycythemia vera (PV) when the WHO and European Clinical and Pathological (WHO-ECMP) criteria are applied. Bone marrow cellularities on histopathology are overlapping in normocellular ET, prodromal PV and PV carrying the JAK2V617F mutation. Increased erythrocyte counts at a cut off level of 5.8x1012/L separates JAK2 mutated ET and prodromal PV from overt PV. JAK2 wild type ET and MF carrying the MPL515 mutation is a distinct MPN entity featured by increase of clustered small and giant megakaryocytes with hyperlobulated stag-horn-like nuclei, in a normal cellular bone marrow without features of PV. JAK2/MPL wild type ET and MF carrying one of the calreticulin (CALR) mutations is the third distinct entity associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation (PMGM) characterized by dense clustered, large, and immature dysmorphic megakaryocytes with bulky (bulbous) hyperchromatic nuclei, which are never seen in JAK2V617F mutated ET, prodromal PV, EMGM and PV, and also not in MPL515 mutated ET and MF.

journal of hematology and blood disorders

Prospective Evaluation of WHO and European Clinical, Molecular and Pathological (WHO-ECMP) Criteria for Myeloproliferative Neoplasms (MPN) of Various Molecular Etiology: Characteristics of JAK2V617F, MPL515 and CALR Mutated MPN