An Introduction to Myeloproliferative Disorders
Myeloproliferative disorders include a number of conditions that are characterised by excessive accumulation of blood cells or platelets and carry a risk of progression to acute leukaemia. The discovery of mutations in the JAK2, MPL and CALR genes has increased our understanding of the pathogenesis of Philadelphia-negative myeloproliferative neoplasms (PV, MF and ET), leading to improved diagnosis and management. However, JAK2 inhibition has not produced long-term disease remission and there is a need for novel therapeutic targets. Emerging approaches include isocitrate dehydrogenase inhibitors, spliceosome inhibitors, pegylated interferon with ruxolitinib and immune checkpoint inhibitors.
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Myeloproliferative Disorders Content
An Exciting New Era in the Treatment of Myeloproliferative Neoplasms
Oncology & Hematology Review, 2016;12(2):71–4 DOI: https://doi.org/10.17925/OHR.2016.12.02.71
Janus kinase/signal transducer and activator of transcription inhibition A major breakthrough in understanding the molecular drivers of myeloproliferative neoplasms (MPNs) came in 2005, with the discovery of the Janus kinase 2 (JAK2) V617F driver mutation.1 Mutated JAK activates a number of downstream pathways implicated in the proliferation and survival of malignant cells, including the signal […]
Proteomics in Myeloproliferative Disorders
European Haematology, 2008;2(1):78-81 DOI: https://doi.org/10.17925/EOH.2008.02.1.78
Myeloproliferative disorders (MPDs) are haematopoietic stem cell malignancies characterised by overproduction of one or more mature myeloid lineage. The term ‘myeloproliferative syndrome’ was first introduced in 1951 by Dameshek,1 who defined chronic myeloid leukaemia (CML), polycythaemia vera (PV), essential thrombo-cythaemia (ET) and idiopathic myelofibrosis (IMF) as a group of closely inter-related neoplasms caused by proliferate […]
Myeloproliferative Neoplasms – Classification, Diagnostic and Therapeutic Options in the Light of Molecular Findings
European Haematology, 2009;3(1):57-60 DOI: https://doi.org/10.17925/EOH.2009.03.1.57
The term myeloproliferative disorders (MPDs) was introduced by Demeshek in 1951.1 He postulated that various clinical conditions, such as chronic granulocytic leukaemia, polycythaemia vera (PV), idiopathic myeloid metaplasia, thrombocythaemia, megacariocytic leukaemia and erythroleukaemia, can be regarded as variable manifestations of proliferative activity of bone marrow cells. In the following few years, the term MPD was […]
Philadelphia-negative Chronic Myeloproliferative Disorders in Children
European Haematology, 2010;4:51-4 DOI: https://doi.org/10.17925/EOH.2010.04.0.51
Polycythaemia vera (PV), essential thrombocythaemia (ET) and primary myelofibrosis (PMF) are classified as Philadelphia-negative chronic myeloproliferative disorders (Ph-MPDs), clonal haematopoietic disorders1,2 that result in overproduction of mature myeloid cells and that are characterised by an increased risk of thrombotic and/or haemorrhagic complications3 and a possible evolution into MF and/or acute leukaemia.4 In PV there is […]
Leukaemic Transformation of Philadelphia-chromosome-negative Myeloproliferative Neoplasms – A Review of the Molecular Background
European Oncology & Haematology, 2011;7(1):59-62 DOI: https://doi.org/10.17925/EOH.2011.07.01.59
Philadelphia-chromosome-negative myeloproliferative neoplasms (MPNs), including polycythaemia vera (PV), primary myelofibrosis (PMF) and essential thrombocythaemia (ET) are defined as clonal haematopoietic stem cell disorders. They are characterised by increased proliferation of terminally differentiated myeloid cells.
Proteomics in Myeloproliferative Disorders
Asia-Pacific Oncology & Haematology, 2008;1(1):77-80
The term ‘proteome’ was proposed by Wilkins et al. in 1995 and referred to the whole set of proteins present in a cell or a biological fluid at a given time. The proteome is a fundamentally dynamic entity that reflects the best functional status of a biological system. There is increasing evidence that the large […]
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