Review
Copyright ©2013 Baishideng. All rights reserved.
World J Hematol. Aug 6, 2013; 2(3): 71-88
Published online Aug 6, 2013. doi: 10.5315/wjh.v2.i3.71
PVSG and WHO vs European Clinical, Molecular and Pathological Criteria for prefibrotic myeloproliferative neoplasms
Jan Jacques Michiels, Zwi Berneman, Wilfried Schroyens, King H Lam, Hendrik De Raeve
Jan Jacques Michiels, Zwi Berneman, Wilfried Schroyens, Department of Hematology, University Hospital Antwerp, Wilrijkstraat 10, B-2650 Edegem, Antwerp, Belgium
Jan Jacques Michiels, European Working Group on Myeloproliferative Neoplasms, Goodheart Institute, Erasmus Tower, Veenmos 13, 3069 AT, Rotterdam, The Netherlands
King H Lam, Department of Pathology, Erasmus University Medical Center Rotterdam, Dr. Molewaterplein 50, 3015 GD Rotterdam, The Netherlands
Hendrik De Raeve, Department of Pathology OLV Hospital Aalst and University Hospital Brussels, OLV Ziekenhuis, Moorselbaan 164, B-9300 Aalst, Belgium
Author contributions: Michiels JJ and De Raeve H designed the study and wrote the manuscript; Michiels JJ, Berneman Z and Schroyens W collected and analysed the clinical features of myeloproliferative neoplasms (MPN); Lam KH and De Raeve H perfomed the pathology studies and interpreted the bone histology diagnotic findings in MPN of various molecular etiology.
Correspondence to: Jan Jacques Michiels, MD, PhD, Senior Internist, European Working Group on Myeloproliferative Neoplasms, Goodheart Institute, Erasmus Tower, Veenmos 13, 3069 AT, Rotterdam, The Netherlands. goodheartcenter@upcmail.nl
Telephone: +31-62-6970534
Received: January 11, 2013
Revised: June 14, 2013
Accepted: June 18, 2013
Published online: August 6, 2013
Core Tip

Core tip: The integrated World Health Organization (WHO) and European Clinical, Molecular and Pathological classification of the myeloproliferative neoplasms include JAK2V617F mutated normocellular essential thrombocythemia (WHO-ET), prodromal polycythemia vera (PV), classical PV, and hypercellular ET due to megakaryocytic, granulocytic myeloproliferation. Evolution of prodromal PV into overt PV is common. JAK2/MPL wild hypercellular ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation is characterized by densely clustered immature dysmorphic megakaryocytes with bulky (bulbous) hyperchromatic nuclei, which are never seen in JAK2V617F mutated ET and PV, and also not in JAK2 wild type normocellular ET (WHO-ET) carrying the MPL515 mutation.