Abstract:
:Transformation to secondary myelofibrosis (MF) occurs as part of the natural history of polycythemia vera (PPV-MF) and essential thrombocythemia (PET-MF). Although primary (PMF) and secondary MF are considered similar diseases and managed similarly, there are few studies specifically focused on the latter. The aim of this study was to characterize the mutation landscape, and describe the main clinical correlates and prognostic implications of mutations, in a series of 359 patients with PPV-MF and PET-MF. Compared with PV and ET, the JAK2V617F and CALR mutated allele burden was significantly higher in PPV-MF and/or PET-MF, indicating a role for accumulation of mutated alleles in the process of transformation to MF. However, neither the allele burden nor the type of driver mutation influenced overall survival (OS), while absence of any driver mutation (triple negativity) was associated with significant reduction of OS in PET-MF, similar to PMF. Of the five interrogated subclonal mutations (ASXL1, EZH2, SRSF2, IDH1, and IDH2), that comprise a prognostically detrimental high molecular risk (HMR) category in PMF, only SRSF2 mutations were associated with reduced survival in PET-MF, and no additional mutation profile with prognostic relevance was highlighted. Overall, these data indicate that the molecular landscape of secondary forms of MF is different from PMF, suggesting that unknown mutational events might contribute to the progression from chronic phase disease to myelofibrosis. These findings also support more extended genotyping approaches aimed at identifying novel molecular abnormalities with prognostic relevance for patients with PPV-MF and PET-MF. Am. J. Hematol. 91:681-686, 2016. © 2016 Wiley Periodicals, Inc.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Rotunno G,Pacilli A,Artusi V,Rumi E,Maffioli M,Delaini F,Brogi G,Fanelli T,Pancrazzi A,Pietra D,Bernardis I,Belotti C,Pieri L,Sant'Antonio E,Salmoiraghi S,Cilloni D,Rambaldi A,Passamonti F,Barbui T,Manfredini R,Cadoi
10.1002/ajh.24377subject
Has Abstractpub_date
2016-07-01 00:00:00pages
681-6issue
7eissn
0361-8609issn
1096-8652journal_volume
91pub_type
杂志文章,多中心研究abstract::Complete remission can be achieved in 60-80% of adults with diffuse aggressive non-Hodgkin's lymphoma. However, 20-40% of them will subsequently relapse. Nevertheless, formal follow-up guidelines for recurrence detection have never been advocated. We analyzed the pattern of relapse in 30 patients with intermediate- an...
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