Primary myelofibrosis is not primary anymore since the discovery of MPL515 and CALR mutations as driver causes of mono-linear megakaryocytic and dual megakaryocytic granulocytic myeloproliferation and secondary myelofibrosis

Abstract

Primary myelofi brosis (PMF) is a distinct clinicopathological myeloproliferatve disease (MPD) not preceded by any other MPD ET, PV, CML, or MDS. With the advent of the MPL 515 , CALR and JAK2 V617F mutations as the driver causes of three variants of ET (thrombocythemias) the term ET is not essential and PMF is not primary anymore simple because myelofi brosis is a secondary event in all three molecular variants of thrombocythemia and PV. CALR-mutated hypercellular thrombocythemia and MPL 515 -mutated normocellular thrombocythemia are two novel distinct myeloproliferative neoplasms (MPN) disease entities without features of PV and complicated by splenomegaly and secondary myelofi brosis during life-long follow-up. The life expectancy of JAK2 V617F , MPL 515 and CALR mutated MPNs are related to allele burden, bone marrow cellularity and the degree of anemia, splenomegaly and myelofi brosis during life-long follow-up. Research ArticlePrimary myelofi brosis is not primary anymore since the discovery of MPL 515 and CALR mutations as driver causes of mono-linear megakaryocytic and dual megakaryocytic granulocytic myeloproliferation and secondary myelofi brosis How to cite this article: Michiels JJ, De Raeve H. Primary myelofi brosis is not primary anymore since the discovery of MPL 515 and CALR mutations as driver causes of mono-linear megakaryocytic and dual megakaryocytic granulocytic myeloproliferation and secondary myelopfi brosis. Int J Bone Marrow Res. 2019; 2: 018-026. https://doi.org/10.29328/journal.ijbmr.1001003 Primary myelofi brosis is not primary anymore since the discovery of MPL 515 and CALR mutations as driver causes of mono-linear megakaryocytic and dual megakaryocytic granulocytic myeloproliferation and secondary myelopfi brosis