Cellular and Molecular Biology



BCR-ABL negative myeloproliferative neoplasia: a review of involved molecular mechanisms

Suzanne M. Koopmans1, Harry C. Schouten2 and AriŽnne M.W. van Marion3

1Department of Pathology of the University Hospital Maastricht, 2Department of Internal Medicine division of Haematology of the University Hospital Maastricht, Maastricht and 3Department of Pathology of the VieCuri Medical Centre, Venlo, The Netherlands

Offprint requests to: S.M. Koopmans, Department of Pathology, University Hospital Maastricht Postbus 5800, 6202 AZ Maastricht, The Netherlands. e-mail: s.koopmans@mumc.nl

Summary. 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 JAK2V617F 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. Histol Histopathol 30, 151-161 (2015)

Key words: Myeloproliferative neoplasia, Essential thrombocythemia, Polycythemia vera, Primary myelofibrosis, JAK2 mutation

DOI: 10.14670/HH-30.151