Changed concepts and definitions of myeloproliferative neoplasms MPN, myelodysplastic syndromes MDS and myelodysplastic-myeloproliferative neoplasms MDS-MPN in the updated 2008 WHO classificationReport as inadecuate




Changed concepts and definitions of myeloproliferative neoplasms MPN, myelodysplastic syndromes MDS and myelodysplastic-myeloproliferative neoplasms MDS-MPN in the updated 2008 WHO classification - Download this document for free, or read online. Document in PDF available to download.

Journal of Hematopathology

, 2:205

First Online: 26 September 2009Received: 28 August 2009Accepted: 03 September 2009

Abstract

The purpose of this overview is to discuss the changes in the 2008 WHO classification of myeloid neoplasms, with exclusion of acute myeloid leukaemia. Specific mutations or rearrangements leading to constitutive activation of growth factor receptors or cytoplasmic tyrosine kinases are now recognised as recurrent genetic events characterising the group of myeloproliferative neoplasms MPN. A newly introduced subgroup consists of patients with persistent eosinophilia and myeloid or lymphoid proliferations harbouring specific genetic changes involving platelet-derived growth factor receptors alpha and beta PDGFRA and PDGFRB or fibroblast growth factor receptor 1 FGFR1. The clinical relevance of recognising myeloid neoplasms with aberrant tyrosine kinase activity is based in novel treatment options with tyrosine kinase inhibitors. The myelodysplastic syndromes MDS without increased blasts are further divided into subtypes of refractory cytopaenias with unilineage dysplasia. A new provisional entity is refractory cytopaenia of childhood. Down syndrome- and therapy-related myeloid neoplasms, including MDS, were moved to the section of acute myeloid leukaemia and related precursor neoplasms.

KeywordsReview Classification Myelodysplastic syndrome Myeloproliferative neoplasm Updated WHO classification  Download fulltext PDF



Author: Konnie M. Hebeda - F. Fend

Source: https://link.springer.com/



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