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.
Highlights
Primary myelofibrosis (PMF) is currently classified with polycythemia vera (PV) and essential thrombocythemia (ET) under the broad World Health Organization (WHO) category of myeloproliferative neoplasms (MPN)[1]
In 2011, cytogenetic information was incorporated into the general framework of dynamic IPSS (DIPSS) in order to develop a more comprehensive DIPSS-plus model[11]
Among the above-listed variables, we focused on driver mutational status, high-risk mutations and karyotype, in order to develop contemporary risk models that included both molecular and cytogenetic information
Summary
Primary myelofibrosis (PMF) is currently classified with polycythemia vera (PV) and essential thrombocythemia (ET) under the broad World Health Organization (WHO) category of myeloproliferative neoplasms (MPN)[1]. IPSS includes five clinically derived risk variables: age >65 years, hemoglobin 25 × 109/l, circulating blasts ≥1% and Tefferi et al Blood Cancer Journal (2018)8:72 constitutional symptoms; the presence of 0, 1, 2, and ≥3 of these adverse features defines the IPSS risk categories of low, intermediate-1, intermediate-2, and high, with corresponding median survivals of 11.3, 7.9, 4, and 2.3 years.
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