title: BCR-ABL negative myeloproliferative neoplasia: a review of involved molecular mechanisms.
abstract: The clonal bone marrow stem cell disorders essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF) belong to the group of Philadelphia chromosome negative myeloproliferative neoplasia (Ph- MPN). In 2005 the JAK2(V617F) mutation was discovered which has generated more insight in the pathogenetic mechanism of the MPNs. More mutations have been detected in MPN patients since. However, the underlying cause of MPN has not been discovered so far. The mechanism of increased angiogenesis in MPNs and the development of fibrosis in the bone marrow in PMF patients and in some ET and PV patients is still not known. This review will focus on the most important molecular pathogenetic mechanisms in MPN patients.
authors.affiliation: Department of Pathology of the University Hospital Maastricht, Maastricht, The Netherlands. s.koopmans@mumc.nl.
authors.forename: Suzanne M
authors.initials: SM
authors.lastname: Koopmans
authors.affiliation: Department of Internal Medicine division of Haematology of the University Hospital Maastricht, Maastricht, The Netherlands.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Schouten
authors.affiliation: Department of Pathology of the VieCuri Medical Centre, Venlo, The Netherlands.
authors.forename: Ariënne M W
authors.initials: AM
authors.lastname: van Marion
mesh_terms: D000818:Animals; D019046:Bone Marrow Neoplasms / Q000235:genetics* / Q000378:metabolism* / Q000473:pathology; D016044:Fusion Proteins, bcr-abl / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D053614:Janus Kinase 2 / Q000235:genetics; D009196:Myeloproliferative Disorders / Q000235:genetics / Q000378:metabolism* / Q000473:pathology
chemical_list: D016044:Fusion Proteins, bcr-abl; C507924:JAK2 protein, human; D053614:Janus Kinase 2