Table 1.
Overview of prognostic scoring systems for ET, PV, and PMF/SMF.
| MPN subtype ⇨ | ET | PV | PMF/SMF | ||||||||||||||
| Risk score ⇨ Parameter ⇩ |
Conventional/ELN [61] | IPSET [62] | IPSET-thrombosis [63] | MIPSS-ET [64] | Conventional/ELN [61] | IPSS for PV [65] | MIPSS-PV [64] | IPSS [13] | DIPSS [66] | DIPSS+ [67] | MIPSS70 [14] | MIPSS70+ [14] | MIPSS70+ v2 [68] | GIPSS [32] | MYSEC (→ sMF) [69] | MTSS (→ transplant) [70] | |
| Age | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | |||||
| Sex | ✓ | ||||||||||||||||
| Clinical | Leukocytes | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | |||||||
| Hemoglobin/RBC | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||
| Blasts in peripheral blood | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||
| Platelets | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||||
| Constitutional symptoms | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||
| Transfusion demand | ✓ | ||||||||||||||||
| Thrombosis history | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | |||||||||||
| Cardiovascular risk factors | ✓ | ||||||||||||||||
| Bone marrow fibrosis | ✓ | ||||||||||||||||
| HLA-mismatched unrelated donor | ✓ | ||||||||||||||||
| Karnofsky performance status | ✓ | ||||||||||||||||
| (Cyto-) Genetic | JAK2V617F present or MPL/CALR absent | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||
| Adverse/HMR mutations | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | ✓ | |||||||||
| Unfavorable karyotype | ✓ | ✓ | ✓ | ✓ | ✓ | ||||||||||||
| VHR karyotype | ✓ | ✓ | ✓ | ||||||||||||||
| Cytogenetic risk variable | ✓ | ||||||||||||||||
| Time of creation (from early to recent): |
|
|
|
* | ** | ||||||||||||
The evolution of prognostic scoring systems in ET (yellow), PV (red), and PMF/SMF (blue) characterized by the incorporated clinical and (cyto-) genetic parameters (✓). The arrows below schematically represent the time of creation from distant past (left) until present (right) and highlight the incorporation of genetic parameters over time. Abbreviations: DIPSS, dynamic IPSS; ELN, European LeukemiaNet; ET, essential thrombocythemia; GIPSS, genetically inspired prognostic scoring system; HLA, human leukocyte antigen; HMR, high molecular risk; IPSET, international prognostic score for ET; IPSS, International Prognostic Scoring System; MIPSS, mutation-enhanced international prognostic scoring system; MTSS, myelofibrosis transplant scoring system; MYSEC, myelofibrosis secondary to PV and ET prognostic model; PMF, primary myelofibrosis; PV, polycythemia vera; RBC, red blood cells; VHR, very high risk; * introduced for sMF (pPV-MF and pET-MF); ** risk score for patients undergoing transplantation.