Myelofibrosis Treatment Algorithm 2018
Open Access
- 31 July 2018
- journal article
- research article
- Published by Springer Science and Business Media LLC in Blood Cancer Journal
- Vol. 8 (8), 72
- https://doi.org/10.1038/s41408-018-0109-0
Abstract
Two novel prognostic systems for primary myelofibrosis (PMF) were recently unveiled: GIPSS (genetically inspired prognostic scoring system) and MIPSS70 (mutation-enhanced international prognostic scoring system for transplant-age patients). GIPSS is based exclusively on genetic markers: mutations and karyotype. MIPSS70 includes mutations and clinical risk factors. In its most recent adaptation, the prognostic value of MIPSS70 has been bolstered by the inclusion of a three-tiered cytogenetic risk stratification and use of hemoglobin thresholds that are adjusted for sex and severity (MIPSS70+ version 2.0). GIPSS features four, MIPSS70 three, and MIPSS70+ version 2.0 five risk categories. MIPSS70 is most useful in the absence of cytogenetic information. MIPSS70+ version 2.0 is more comprehensive than MIPSS70 and is the preferred model in the presence of cytogenetic information. Both MIPSS70 and MIPSS70+ version 2.0 require an online score calculator (http://www.mipss70score.it). GIPPS offers a lower complexity prognostic tool that reliably identifies candidates for allogeneic stem cell transplant (GIPSS high-risk disease) or long-term observation with little or no therapeutic intervention (GIPSS low-risk disease). Ultimately, we favor a step-wise prognostication approach that starts with GIPSS but also considers MIPSS70+ version 2.0 for confirming the most appropriate treatment approach for the individual patient.Keywords
This publication has 38 references indexed in Scilit:
- Presentation and outcome of patients with 2016 WHO diagnosis of prefibrotic and overt primary myelofibrosisBlood, 2017
- Targeted deep sequencing in primary myelofibrosisBlood Advances, 2016
- The prognostic impact of bone marrow fibrosis in primary myelofibrosisAmerican Journal of Hematology, 2016
- Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosisBlood, 2014
- Mutations and prognosis in primary myelofibrosisLeukemia, 2013
- Circulating Interleukin (IL)-8, IL-2R, IL-12, and IL-15 Levels Are Independently Prognostic in Primary Myelofibrosis: A Comprehensive Cytokine Profiling StudyJournal of Clinical Oncology, 2011
- Safety and Efficacy of INCB018424, a JAK1 and JAK2 Inhibitor, in MyelofibrosisNew England Journal of Medicine, 2010
- A dynamic prognostic model to predict survival in primary myelofibrosis: a study by the IWG-MRT (International Working Group for Myeloproliferative Neoplasms Research and Treatment)Blood, 2010
- JAK2 germline genetic variation affects disease susceptibility in primary myelofibrosis regardless of V617F mutational status: nullizygosity for the JAK2 46/1 haplotype is associated with inferior survivalLeukemia, 2009
- New prognostic scoring system for primary myelofibrosis based on a study of the International Working Group for Myelofibrosis Research and TreatmentBlood, 2009