Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12‐year study

This study examined mortality rates among 2475 patients with thalassaemia and sickle cell disease (SCD) per year over 12 years in Greece, recording 335 deaths (27.92/year), with an overall mortality rate of 1.13% per year. The primary aim was to identify causes of death, comorbidities, treatment efficacy and iron overload prevalence. Of the deaths, 239 were attributed to thalassaemia and 96 to SCD. For thalassaemia patients, cardiac failure, hepatocellular carcinoma and sepsis were the leading causes of death, with no neoplasms observed in β+/β+ genotypes. In SCD, sepsis, liver failure and stroke were the predominant causes, with sepsis-related deaths higher in frequently transfused patients. The median age of death was significantly lower in thalassaemia (50 years) compared to SCD (58.49 years, p < 0.001). Differences in comorbidities and treatment effectiveness highlight the need for improved management strategies. Addressing iron overload, optimizing chelation therapy and expanding hydroxyurea use in SCD patients could enhance survival and quality of life. Strengthening treatment protocols and monitoring may reduce mortality, emphasizing the importance of targeted interventions in haemoglobinopathies.

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Publication Details

Journal
British Journal of Haematology
Published
2025-03-16
DOI
https://doi.org/10.1111/bjh.20043
Citations
4
Primary Topic
Hemoglobinopathies and Related Disorders
Type
article
Field-Weighted Citation Impact
6.50
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article

Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12‐year study

Loukia Evliati, Artemis Vasiliadi, Chrysoula Kalkana, Sophia Delicou et al.
4 citations
British Journal of Haematology
Hemoglobinopathies and Related Disorders
6.50
article

Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients: A 12‐year study

Loukia Evliati, Artemis Vasiliadi, Chrysoula Kalkana, Sophia Delicou, Panagiota Giasari, Michael D. Diamantidis, Eleni Kapsali, Evangelos Klironomos, D. Vini, Despina Papadopoulou, Ch. Zisis, Theodora Maria Venou, Ioannis Lafiatis, Anastasia Serpanou, Antonis Kattamis, Foteini Petropoulou, Sofia Tsagia, Marianna Katsatou, Konstantinos Manganas, Alexandra Kouraklis, Aikaterini Xydaki, Konstantina Papaioannou, Efthymia Vlachaki, Vasiliki Pappi, Stavroula Kyriakaki, Polyxeni Delaporta, Christos Chatzoulis, Maria P. Drandaki, Despoina Pantelidou, Stylianos Lafioniatis, Anastasia Goula, Angelos Gkoutzouvelidis, Eirini Spachiou
article en
4 citations

Abstract

This study examined mortality rates among 2475 patients with thalassaemia and sickle cell disease (SCD) per year over 12 years in Greece, recording 335 deaths (27.92/year), with an overall mortality rate of 1.13% per year. The primary aim was to identify causes of death, comorbidities, treatment efficacy and iron overload prevalence. Of the deaths, 239 were attributed to thalassaemia and 96 to SCD. For thalassaemia patients, cardiac failure, hepatocellular carcinoma and sepsis were the leading causes of death, with no neoplasms observed in β+/β+ genotypes. In SCD, sepsis, liver failure and stroke were the predominant causes, with sepsis-related deaths higher in frequently transfused patients. The median age of death was significantly lower in thalassaemia (50 years) compared to SCD (58.49 years, p < 0.001). Differences in comorbidities and treatment effectiveness highlight the need for improved management strategies. Addressing iron overload, optimizing chelation therapy and expanding hydroxyurea use in SCD patients could enhance survival and quality of life. Strengthening treatment protocols and monitoring may reduce mortality, emphasizing the importance of targeted interventions in haemoglobinopathies.

British Journal of HaematologyVol. 206(5)
Democritus University of Thrace (GR), University of Ioannina (GR), National and Kapodistrian University of Athens (GR), AHEPA University Hospital (GR), Agios Pavlos General Hospital (GR), General University Hospital of Patras (GR), General Hospital of Athens G. Genimatas (GR), University Hospital of Larissa (GR), Ippokrateio General Hospital of Thessaloniki (GR), Children's Hospital Agia Sophia (GR), Corfu General Clinic (GR), The General Hospital of Heraklion "Venizeleio-Pananio" (GR), Chania General Hospital ”St. George” (GR), General Hospital of Nikea (GR), KAT General Hospital of Attica (GR), University Hospital of Alexandroupolis (GR), Evangelismos Hospital (GR), Technological Educational Institute of Eastern Macedonia and Thrace (GR)
Good health and well-being
Openalex Percentile: Top 4%
Hemoglobinopathies and Related Disorders
6.50
DOIOpen Access PDFOpenAlex
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