Journal of Hematology & Thromboembolic Diseases
Open Access
ISSN: 2329-8790
+44 7868 792050
ISSN: 2329-8790
+44 7868 792050
Francisca Valster
England
Review Article
Secondary Myelofibrosis in the Naturel History of JAK2, MPL and CALR Mutated Myeloproliferative Neoplasms
Author(s): Jan Jacques Michiels, Francisca Valster, Vincent Potter, Katrien Schelfout, Wilfried Schroyens, Karel Fostier, Timothy Devos, Fibo Ten Kate and Hendrik De RaeveJan Jacques Michiels, Francisca Valster, Vincent Potter, Katrien Schelfout, Wilfried Schroyens, Karel Fostier, Timothy Devos, Fibo Ten Kate and Hendrik De Raeve
The WHO classification of the Myeloprolferative Neoplasms (MPN) distinguishes Essential Thrombocythemia (ET), Polycythemia Vera (PV) and Primary Myelofibrosis (PMF). Myelofibrosis (MF) is not a disease because reticulin and collagen fibrosis are produced by polyclonal fibroblasts in response to cytokines released from the clonal granulocytic and megakaryocytic progenitor cells in myeloproliferative disorders (MPD) in patients with ET and PV. The Hannover and Cologne Bone Marrow classification defined chronic or primary megakaryocytic granulocytic myeloproliferation (PMGM) as the third distinct prefibrotic MPN without features of PV. Vainchenker (2005) discovered the JAK2V617F somatic mutation as the driver cause of ET, trilinear PV of erythrocytic, megakaryocytic and granulocytic myeloproliferation (EMGM) and myeloid neoplasia of the spleen with secondary MF. Prefibrotic JAK2V617F mutate.. View More»
DOI:
10.4172/2329-8790.1000238