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Oxford Specialist Handbook: Myeloproliferative Neoplasms最新文献

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Essential thrombocythaemia 基本thrombocythaemia
Oxford Specialist Handbook: Myeloproliferative Neoplasms Pub Date : 2020-07-01 DOI: 10.1093/med/9780198744214.003.0010
C. Besses, B. Bellosillo, A. Álvarez-Larrán, T. Mughal
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引用次数: 0
Polycythaemia vera
Oxford Specialist Handbook: Myeloproliferative Neoplasms Pub Date : 2020-07-01 DOI: 10.1093/med/9780198744214.003.0008
T. Barbui, T. Mughal, G. Finazzi
{"title":"Polycythaemia vera","authors":"T. Barbui, T. Mughal, G. Finazzi","doi":"10.1093/med/9780198744214.003.0008","DOIUrl":"https://doi.org/10.1093/med/9780198744214.003.0008","url":null,"abstract":"Polycythaemia vera (PV) is a clonal stem cell disorder characterized by erythrocytosis and associated with burdensome symptoms, risk of thrombohaemorrhagic complications, and transformation to myelofibrosis and acute myeloid leukaemia. Diagnostic criteria are very recently revised by the World Health Organization (WHO) based on haemoglobin and haematocrit levels, bone marrow morphology consistent with trilineage proliferation and presence of the JAK2 V617 mutation. Cytoreductive therapy is indicated in patients at increased risk of thrombosis. Hydroxyurea (HU) remains the most commonly used first-line cytoreductive therapy and interferon (IFN) is used either at failure of HU or in selected patients as first-line therapy. A recent phase 3 trial has shown the superiority of the JAK1/2 inhibitor ruxolitinib in comparison to best available treatment in HU-intolerant or resistant patients.","PeriodicalId":262404,"journal":{"name":"Oxford Specialist Handbook: Myeloproliferative Neoplasms","volume":"23 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129996210","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Systemic mastocytosis
Oxford Specialist Handbook: Myeloproliferative Neoplasms Pub Date : 2020-07-01 DOI: 10.1093/med/9780198744214.003.0011
J. Gotlib
{"title":"Systemic mastocytosis","authors":"J. Gotlib","doi":"10.1093/med/9780198744214.003.0011","DOIUrl":"https://doi.org/10.1093/med/9780198744214.003.0011","url":null,"abstract":"The study of systemic mastocytosis (SM) is undergoing dynamic change. Understanding of the biologic underpinnings of the disease, including its various World Health Organization-defined subtypes, is being catalysed by next generation sequencing in conjunction with sophisticated molecular biology tools which have allow refined analysis of mast cell immunophenotypes and intracellular signalling networks. In turn, this has provided translational opportunities for identifying new targets of therapy and biologic correlates of response, particularly in advanced forms of disease. Because SM is a rare and heterogeneous disease, collaborative efforts are being undertaken to aggregate data from large numbers of patients to better define the natural history and prognosis of its variants. This chapter aims to summarize the major features of mast cell biology, diagnosis, classification, and treatment, including a focus on more recent developments regarding the mutational landscape of SM and novel therapeutics.","PeriodicalId":262404,"journal":{"name":"Oxford Specialist Handbook: Myeloproliferative Neoplasms","volume":"73 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2020-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126151059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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