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Current myeloproliferative neoplasm scoring systems for clinical practice.

Hélène Pasquer^1,2, Jean-Jacques Kiladjian^1,3, Lina Benajiba^1,2

¹Centre d'Investigations Cliniques, INSERM CIC 1427, Hôpital Saint-Louis, Université Paris Cité, Assistance Publique des Hôpitaux de Paris, Paris, France.

|October 30, 2024

Summary

Accurate risk stratification is crucial for managing myeloproliferative neoplasms (MPNs). This review compares MPN scoring systems to guide clinical decisions and improve patient outcomes.

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Area of Science:

Hematology
Oncology
Genetics

Background:

Myeloproliferative neoplasms (MPNs) are clonal myeloid malignancies driven by JAK-STAT pathway mutations.
MPN management balances risk assessment for disease evolution and quality of life.
Risk factors vary by MPN subtype: myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET).

Purpose of the Study:

To provide an overview of MPN disease evolution-associated event incidence.
To conduct a comparative review of available scoring systems for MPN risk stratification.
To propose an algorithm for clinical application of MPN risk scores.

Main Methods:

Literature review of MPN disease evolution and risk scoring systems.
Comparative analysis of scoring systems based on MPN subtype and predicted outcomes.
Development of a clinical algorithm for score selection.

Main Results:

Myelofibrosis patients have poorer prognosis; hematopoietic stem cell transplantation (HSCT) is curative but high-risk.
Polycythemia vera and essential thrombocythemia management prioritizes vascular event prediction.
Numerous scoring systems exist, complicating clinical choice due to MPN heterogeneity.

Conclusions:

Accurate risk stratification is essential for optimizing MPN management and therapeutic strategies.
A comparative review and proposed algorithm can aid clinicians in selecting appropriate risk scores.
Improved risk assessment facilitates personalized treatment and better patient outcomes in MPNs.