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This Article Full Text Full Text (PDF) CME: Take the course for this article: Advances in Understanding and Management of Myeloproliferative Neoplasms FREE NURSING CONTACT HOURS All Versions of this Article: caac.20009v1 59/3/171    most recent Submit a letter to the editor View responses Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Vannucchi, A. M. Articles by Tefferi, A. Search for Related Content PubMed PubMed Citation Articles by Vannucchi, A. M. Articles by Tefferi, A.

Advances in Understanding and Management of Myeloproliferative Neoplasms

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;59:171–191. © 2009 American Cancer Society, Inc.

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