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MPN risk scoring: Current algorithms for clinical practice
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Myeloproliferative neoplasms (MPN), including essential thrombocythemia (ET), myelofibrosis (MF), and polycythemia vera (PV), present varying risks that impact patient outcomes.1 Disease management recommendations aim to prevent disease evolution, whilst maximizing patients’ quality of life.1 Several risk stratification scoring systems have emerged to predict disease evolution, with recent models incorporating molecular data for improved accuracy.1 The availability of numerous systems along with substantial disease heterogeneity and a high clinical need for outcome predictions can create challenges for clinicians in selecting an optimal system.1 To address this, Pasquer et al.1 conducted a comparative review of currently available MPN scoring systems and proposed an up-to-date algorithm to optimize MPN patient prognostication in the clinic, which was recently published in Blood. |
| Key learnings |
| Findings from this literature review led to the proposal of new algorithms for using MPN scoring systems in clinical practice, with specific algorithms generated for MF, PV, and ET. |
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For MF, OS estimation is essential to tailor therapeutic strategies. Systems like MIPSS70+ v2.0 improve OS predictions, assisting in HSCT decisions. For patients aged >70 years with PMF or SMF and karyotype data, MIPSS70+ v2.0 is preferred; without cytogenetic data, MIPSS70 is recommended. MIPSS70+ v2.0 is also applicable for patients aged >70 years with MF and molecular data. For patients without molecular data, DIPSS-plus or MYSEC-PM is suggested for SMF, while DIPSS or DIPSS-plus is used for PMF, contingent on available cytogenetic data. |
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For PV and ET, evaluation of thrombotic event risk should guide cytoreductive and antiplatelet treatment. If TET2/DNMT3A mutational status is available, ARTS is the preferred thrombotic scoring system for arterial event prediction. If molecular data are unavailable, arterial thrombotic risk evaluation should be evaluated with IPSET-revised risk stratification for ET and two-tiered conventional risk stratification for PV. For venous thrombosis, VETS and IPSET-revised risk stratification should be used for ET, and VETS and two-tiered conventional risk stratification for PV. VETS and ARTS scores should be externally validated prior to their implementation in clinical practice. |
| Using these models, clinicians can better assess patient-specific risks, optimize treatment decisions, and potentially enhance outcomes, particularly through personalized therapeutic strategies. |
Abbreviations: AML, acute myeloid leukemia; ARTS, arterial thrombosis score; ET, essential thrombocytopenia; HSCT, hematopoietic stem cell transplantation; MF, myelofibrosis; MPN, myeloproliferative neoplasm; OS, overall survival; PMF, primary myelofibrosis; PV, polycythemia vera; SMF, secondary myelofibrosis; VETS, venous thrombosis score.
References
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