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Published online before print April 15, 2009
CA Cancer J Clin 2009
doi: 10.3322/caac.20009
© 2009 American Cancer Society
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Advances in Understanding and Management of Myeloproliferative Neoplasms
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Advances in understanding and management of myeloproliferative neoplasms

Alessandro M. Vannucchi, MD1, Paola Guglielmelli, MD2 and Ayalew Tefferi, MD3

1Associate Professor of Hematology, Department of Hematology, University of Florence, Florence, Italy
2Research Fellow at the Department of Hematology, University of Florence, Florence, Italy
3Professor of Medicine and Hematology, Mayo Clinic College of Medicine, Rochester, NY

Corresponding authors: Alessandro M. Vannucchi, MD, Hematology Unit, Dip. Area Critica, University of Florence, Viale Morgagni 85, 50134 Florence, Italy; amvannucchi{at}unifi.it and Ayalew Tefferi, MD, Division of Hematology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905; tefferi.ayalew{at}mayo.edu

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DISCLOSURES: This study was supported by Associazione Italiana per la Ricerca sul Cancro, Milano; Istituto Toscano Tumori; MIUR (COFIN 2006067001_003). The authors report no conflicts of interest.

According to the 2008 World Health Organization classification system for hematologic malignancies, the myeloproliferative neoplasms (MPN) include chronic myelogenous leukemia, polycythemia vera, essential thrombocythemia, primary myelofibrosis, mastocytosis, chronic eosinophilic leukemia-not otherwise specified, chronic neutrophilic leukemia, and "MPN, unclassifiable." All of these clinicopathologic entities are characterized by stem cell-derived clonal myeloproliferation, and their phenotypic diversity is ascribed to the occurrence of distinct oncogenic events. In the last 4 years, new JAK2 and MPL mutations have been added to previously described ABL and KIT mutations as molecular markers of disease in MPN. These discoveries have markedly simplified the approach to clinical diagnosis and have also provided molecular targets for the development of small-molecule drugs. In the current article, the authors provide a clinically oriented overview of MPNs in terms of their molecular pathogenesis, classification, diagnosis, and management. CA Cancer J Clin 2009. © 2009 American Cancer Society, Inc.




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eLetters:

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Estrogen Receptor Might be an Important Factor for Myeloproliferative Neoplasms
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CA Online, 28 Jul 2009 [Full text]



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