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Leukemia. 2018 Jul;32(7):1631-1642. doi: 10.1038/s41375-018-0107-z. Epub 2018 Mar 23.

GIPSS: genetically inspired prognostic scoring system for primary myelofibrosis.

Author information

1
Divisions of Hematology, Departments of Internal Medicine and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA. tefferi.ayalew@mayo.edu.
2
Department of Experimental and Clinical Medicine, CRIMM, Center Research and Innovation of Myeloproliferative Neoplasms, Azienda Ospedaliera Universitaria Careggi, University of Florence, Florence, Italy.
3
Divisions of Hematology, Departments of Internal Medicine and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.
4
Divisions of Hematopathology, Departments of Internal Medicine and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.
5
Divisions of Laboratory Genetics and Genomics, Departments of Internal Medicine and Laboratory Medicine, Mayo Clinic, Rochester, MN, USA.

Abstract

International collaborations over the years have produced a series of prognostic models for primary myelofibrosis (PMF), including the recently unveiled mutation-enhanced international prognostic scoring systems for transplant-age patients (MIPSS70 and MIPSS70-plus). In the current study, we considered the feasibility of a genetically inspired prognostic scoring system (GIPSS) that is exclusively based on genetic markers. Among 641 cytogenetically annotated patients with PMF and informative for previously recognized adverse mutations, multivariable analysis identified "VHR" karyotype, "unfavorable" karyotype, absence of type 1/like CALR mutation and presence of ASXL1, SRSF2, or U2AF1Q157 mutation, as inter-independent predictors of inferior survival; the respective HRs (95% CI) were 3.1 (2.1-4.3), 2.1 (1.6-2.7), 2.1 (1.6-2.9), 1.8 (1.5-2.3), 2.4 (1.9-3.2), and 2.4 (1.7-3.3). Based on HR-weighted risk points, a four-tiered GIPSS model was devised: low (zero points; n = 58), intermediate-1 (1 point; n = 260), intermediate-2 (2 points; n = 192), and high (≥3 points; n = 131); the respective median (5-year) survivals were 26.4 (94%), 8.0 (73%), 4.2 (40%), and 2 (14%) years; the model was internally validated by bootstrapping and its predictive accuracy was shown to be comparable to that of MIPSS70-plus. GIPPS offers a low-complexity prognostic tool for PMF that is solely dependent on genetic risk factors and, thus, forward-looking in its essence.

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