Michiels JJ, Valster F, Wielenga J, Schelfout K, Raeve HD. European vs 2015-World Health Organization clinical molecular and pathological classification of myeloproliferative neoplasms. World J Hematol 2015; 4(3): 16-53 [DOI: 10.5315/wjh.v4.i3.16]
Corresponding Author of This Article
Jan Jacques Michiels, MD, PhD, Investigator, Senior Internist, International Hematology and Bloodcoagulation Research Center, Goodheart Institute and Foundation in Nature Medicine, European Working Group on Myeloproliferative Neoplasms, Erasmus Tower, Veenmos 13, 3069 AT Rotterdam, The Netherlands. goodheartcenter@upcmail.nl
Research Domain of This Article
Hematology
Article-Type of This Article
Review
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Hematol. Aug 6, 2015; 4(3): 16-53 Published online Aug 6, 2015. doi: 10.5315/wjh.v4.i3.16
European vs 2015-World Health Organization clinical molecular and pathological classification of myeloproliferative neoplasms
Jan Jacques Michiels, Fransje Valster, Jenne Wielenga, Katrien Schelfout, Hendrik De Raeve
Jan Jacques Michiels, International Hematology and Bloodcoagulation Research Center, Goodheart Institute and Foundation in Nature Medicine, European Working Group on Myeloproliferative Neoplasms, 3069 AT Rotterdam, The Netherlands
Fransje Valster, Department of Internal Medicine, BRAVIS Hospital, 4624 VT Bergen op Zoom, The Netherlands
Jenne Wielenga, Department of Internal Medicine, Admiraal De Ruyter Hospital, 4382 EE Vlissingen, The Netherlands
Katrien Schelfout, Department of Pathology, BRAVIS Hospital, 4624 VT Bergen op Zoom, The Netherlands
Hendrik De Raeve, Department of Pathology OLV Hospital Aalst OLV Ziekenhuis, B-9300 Aalst, Belgium
Hendrik De Raeve, Department of Pathology, University Hospital, B-1090 Brussels, Belgium
Author contributions: Michiels JJ and De Raeve H designed the study and wrote the manuscript; Michiels JJ, Valster F and Wielenga J collected the clinical and molecular features of myeloproliferative neoplasms (MPN); Schelfout K and De Raeve H perfomed the pathology studies and interpreted the bone histology diagnostic findings in MPN of various molecular etiology.
Conflict-of-interest statement: The authors declare no conflicts of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Jan Jacques Michiels, MD, PhD, Investigator, Senior Internist, International Hematology and Bloodcoagulation Research Center, Goodheart Institute and Foundation in Nature Medicine, European Working Group on Myeloproliferative Neoplasms, Erasmus Tower, Veenmos 13, 3069 AT Rotterdam, The Netherlands. goodheartcenter@upcmail.nl
Telephone: +31-62-6970534
Received: June 23, 2014 Peer-review started: June 23, 2014 First decision: August 14, 2014 Revised: March 14, 2015 Accepted: April 28, 2015 Article in press: April 30, 2015 Published online: August 6, 2015 Processing time: 422 Days and 16.5 Hours
Core Tip
Core tip: The 2015 World Health Organization-Clinical Molecular and Pathological criteria define three phenotypes of JAK2V617F mutated myeloproliferative neoplasms (MPNs) essential thrombocythemia (ET), prodromal polycythemia vera (PV), prodromal PV, hypercellular megakaryocytic-granulocytic myeloproliferation and classical PV vs the JAK2 exon 12 mutated idiopathic eryhrocythemia and PV. MPL515 mutated JAK2 wild type ET and myelofibrosis is a distinct thrombocythemia without features of PV in blood and bone marrow. Calreticulin mutated JAK2/MPL wild type ET and myelofibrosis is the third thrombocythemia entity with characteristic features of primary megakaryocytic granulocytic myeloproliferation in the bone marrow, which are not seen in JAK2 and MPL mutated MPNs. MPN disease burden is best reflected by the degree of anemia and splenomegaly on top of mutation allele burden, bone marrow cellularity and increase of reticulin fibrosis.
