Advances in understanding and management of myeloproliferative neoplasms.
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.
Vannucchi
Alessandro M
AM
Department of Hematology, University of Florence, Florence, Italy. amvannucchi@unifi.it
Guglielmelli
Paola
P
Tefferi
Ayalew
A
D000970:Antineoplastic Agents / Q000627:therapeutic use; D014408:Biomarkers, Tumor; D004252:DNA Mutational Analysis; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D053614:Janus Kinase 2 / Q000037:antagonists & inhibitors / Q000235:genetics; D009196:Myeloproliferative Disorders / Q000145:classification / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy; D018962:Phlebotomy; D011379:Prognosis; D053628:Receptors, Thrombopoietin / Q000235:genetics
D000970:Antineoplastic Agents; D014408:Biomarkers, Tumor; D053628:Receptors, Thrombopoietin; C082218:MPL protein, human; C507924:JAK2 protein, human; D053614:Janus Kinase 2