@article {244661,
	title = {Development of a Thalassemia International Prognostic Scoring System (TIPSS).},
	journal = {Blood cells, molecules \& diseases},
	volume = {99},
	year = {2023},
	pages = {102710},
	abstract = {<p>A prognostic scoring system that can differentiate β-thalassemia patients based on mortality risk is lacking. We analysed data from 3145 β-thalassemia patients followed through a retrospective cohort design for the outcome of death. An a priori list of prognostic variables was collected. β Coefficients from a multivariate cox regression model were used from a development dataset (n~=~2516) to construct a formula for a Thalassemia International Prognostic Scoring System (TIPSS) which was subsequently applied to a validation dataset (n~=~629). The median duration of observation was 10.0~years. The TIPSS score formula was constructed as exp (1.4~{\texttimes}~heart disease + 0.9~{\texttimes}~liver disease + 0.9~{\texttimes}~diabetes + 0.9~{\texttimes}~sepsis + 0.6~{\texttimes}~alanine aminotransferase >=42~IU/L~+~0.6~{\texttimes}~hemoglobin <=9~g/dL~+~0.4~{\texttimes}~serum ferritin >=1850~ng/mL). TIPSS score thresholds of greatest differentiation were assigned as \&lt;2.0 (low-risk), 2.0 to \&lt;5.0 (intermediate-risk), and >=5.0 (high-risk). The TIPSS score was a good predictor for the outcome of death in the validation dataset (AUC: 0.722, 95\%CI: 0.641-0.804) and survival was significantly different between patients in the three risk categories (P~\&lt;~0.001). Compared to low-risk patients, the hazard ratio for death was 2.778 (95\%CI: 1.335-5.780) in patients with intermediate-risk and 6.431 (95\%CI: 3.151-13.128) in patients with high-risk. This study provides a novel tool to support mortality risk categorization for patients with β-thalassemia that could help management and research decisions.</p>
},
	author = {Vitrano, Angela and Musallam, Khaled M and Meloni, Antonella and Karimi, Mehran and Shahina Daar and Ricchi, Paolo and Costantini, Silvia and Vlachaki, Efthymia and Di Marco, Vito and Amal El-Beshlawy and Hajipour, Mahmoud and Ansari, Saqib Hussain and Filosa, Aldo and Ceci, Adriana and Singer, Sylvia Titi and Naserullah, Zaki A and Pepe, Alessia and Cademartiri, Filippo and Pollina, Sebastiano Addario and Scondotto, Salvatore and Dardanoni, Gabriella and Bonifazi, Fedele and Sankaran, Vijay G and Vichinsky, Elliott and Taher, Ali T and Maggio, Aurelio}
}
