Survival and thalassaemia-related complications in HbE/beta-thalassaemia and alpha-thalassaemia: A 10-year longitudinal study in Thailand.

Despite advancements in thalassaemia care, survival rates and complications vary significantly by genotype. This study assesses survival outcomes and associated complications in patients with thalassaemia in north-eastern Thailand. A longitudinal cohort study from October 2012 to September 2023 involved patients aged ≥10 years (median: 31 years, range: 20-74) attending Srinagarind and Udonthani Hospitals. Cox regression analyses identified predictors of survival and complications. Of 380 enrolled patients, 340 completed follow-up. Over 10 years, 39 patients (11.4%) died, primarily from cardiovascular complications (61.5%), at a median age of 45 years (range: 22-72). Patients were grouped as HbE/β-thalassaemia, HbH/HbHCS with HbE mutation and HbH/HbHCS. Survival at 60 years was lower in HbE/β-thalassaemia (55.9%) and HbH/HbHCS with HbE mutation (58.3%) compared to HbH/HbHCS (79.6%, p = 0.08). Significant predictors of mortality included cardiovascular complications (HR 2.4; 95% CI, 1.01-5.5; p = 0.04), recurrent bacterial infections (HR 7.6; 95% CI, 3.1-18.8; p < 0.001) and elevated ferritin (>2500 ng/mL; HR 2.0; 95% CI, 1.01-3.9; p = 0.04). Cardiovascular complications, recurrent bacterial infections and high ferritin levels significantly influence survival in thalassaemia patients. Deletional alpha-thalassaemia patients had superior survival, while HbE/β-thalassaemia and non-deletional alpha-thalassaemia or co-inherited HbE mutation had poorer outcomes, underscoring the need for genotype-specific management strategies.