Triple A Plus (AAA ⁺) Survival Prediction Model for Essential Thrombocythemia: Analysis Involving 7308 Patients

Area of Science:

• Hematology
• Oncology
• Prognostic Modeling

Background:

• Essential thrombocythemia (ET) survival prediction relies on International Prognostic Scoring System (IPSET) and triple-A (AAA) scores.
• The AAA score uses age, absolute neutrophil count (ANC), and absolute lymphocyte count (ALC) as risk factors.
• Need for improved risk stratification in ET patients to guide treatment and predict outcomes.

Purpose of the Study:

• To identify risk variables for ET survival that are independent of the AAA score.
• To develop and validate a new, enhanced prognostic score (AAA+) for ET.
• To compare the predictive performance of the new AAA+ score against existing IPSET and AAA models.

Main Methods:

• Analysis of a Mayo Clinic discovery cohort (658 ET patients) to identify AAA-independent risk variables.
• Multivariable analysis to determine hazard ratios (HRs) for identified risk factors.
• Development of a HR-weighted scoring system (AAA+) and validation in external cohorts from Israel and Italy.

Main Results:

• Identified AAA-independent risk factors: age >70 years, age 50-70 years, elevated ANC, low ALC, elevated absolute monocyte count (AMC), male sex, arterial hypertension, and arterial thrombosis.
• The AAA+ score demonstrated superior time-dependent predictive performance (AUC 0.92/0.91 at 20/25 years) compared to AAA (0.86/0.86) and IPSET (0.81/0.84).
• Validation in external cohorts confirmed the AAA+ model's predictive accuracy.

Conclusions:

• Host-related factors and complete blood count parameters are key determinants of longevity in ET.
• The novel AAA+ risk score provides a more accurate and comprehensive survival prediction for ET patients.
• The AAA+ model enables refined risk stratification, potentially leading to personalized management strategies for ET.