Abstract

Immediately following the 2010 annual American Society of Hematology (ASH) meeting, the 5th International Post-ASH Symposium on Chronic Myelogenous Leukemia and BCR-ABL1-Negative Myeloproliferative Neoplasms (MPNs) took place on 7–8 December 2010 in Orlando, Florida, USA. During this meeting, the most recent advances in laboratory research and clinical practice, including those that were presented at the 2010 ASH meeting, were discussed among recognized authorities in the field. The current paper summarizes the proceedings of this meeting in BCR-ABL1-negative MPN. We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-α, midostaurin and cladribine. In addition, the new ‘Dynamic International Prognostic Scoring System (DIPSS)-plus' prognostic model for primary myelofibrosis (PMF) and the clinical relevance of distinguishing essential thrombocythemia from prefibrotic PMF are discussed.

Highlights

  • The World Health Organization (WHO) classification system for myeloid malignancies uses morphology, in combination with cytochemical, immunophenotypic, cytogenetic and molecular data, to classify myeloid malignancies into five major categories: acute myeloid leukemia (AML), myelodysplastic syndromes (MDSs), myeloproliferative neoplasms (MPNs), MDS/Myeloproliferative Neoplasms (MPNs), and PDGFR- or FGFR1-rearranged myeloid/lymphoid neoplasms associated with eosinophilia.[1]

  • We provide a detailed overview of new mutations with putative epigenetic effects (TET oncogene family member 2 (TET2), additional sex comb-like 1 (ASXL1), isocitrate dehydrogenase (IDH) and enhancer of zeste homolog 2 (EZH2)) and an update on treatment with Janus kinase (JAK) inhibitors, pomalidomide, everolimus, interferon-a, midostaurin and cladribine

  • The World Health Organization (WHO) classification system for myeloid malignancies uses morphology, in combination with cytochemical, immunophenotypic, cytogenetic and molecular data, to classify myeloid malignancies into five major categories: acute myeloid leukemia (AML), myelodysplastic syndromes (MDSs), myeloproliferative neoplasms (MPNs), MDS/MPN, and PDGFR- or FGFR1-rearranged myeloid/lymphoid neoplasms associated with eosinophilia.[1]

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Summary

Published Version Citable link Terms of Use

Tefferi, A.O., Abdel-Wahab, F. Cervantes, J.D. Crispino, G. Finazzi, F. Girodon, H. Gisslinger, et al 2011. Mutations with epigenetic effects in myeloproliferative neoplasms and recent progress in treatment: proceedings from the 5th international post-ash symposium. Blood Cancer Journal 1(3): e7. This article was downloaded from Harvard University’s DASH repository, and is made available under the terms and conditions applicable to Other Posted Material, as set forth at http:// nrs.harvard.edu/urn-3:HUL.InstRepos:dash.current.terms-ofuse#LAA

MEETING REPORT
Introduction
Pathogenetic relevance
New mutations in MPNs with putative epigenetic effect
The largest study of IDH mutation analysis in MPN involved
JAK inhibitor treatment trials
Other treatment trials in myelofibrosis
Treatment for mastocytosis
Conclusions
Evidence that essential thrombocythemia is a clonal disorder with
Distinct clinical and biological features of de novo acute myeloid
Gisslinger H et al Bone marrow fibrosis and diagnosis of
Contentin N et al Prognostic impact of isocitrate dehydrogenase
Findings
Treatment of primary proliferative polycythaemia by venesection
Full Text
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