MPN risk scoring: Current algorithms for clinical practice

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.

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 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.