ISSN: 2329-8790
+44 1478 350008
Karel Fostier
Belgium
Review Article
Secondary Myelofibrosis in the Naturel History of JAK2, MPL and CALR Mutated Myeloproliferative Neoplasms
Author(s): Michiels JJ, Valster F, Potter V, Schelfout K, Schroyens W, et al.
Michiels JJ, Valster F, Potter V, Schelfout K, Schroyens W, et al.
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