Comparative analysis of mortality patterns and treatment strategies in thalassaemia and sickle cell disease patients:...

Area of Science:

• Hematology
• Public Health
• Genetics

Background:

• Thalassaemia and sickle cell disease (SCD) are inherited blood disorders with significant mortality.
• Effective management strategies are crucial for improving patient outcomes and survival rates.

Purpose of the Study:

• To analyze mortality rates and causes of death in Greek patients with thalassaemia and SCD over 12 years.
• To identify key comorbidities, treatment efficacy, and the prevalence of iron overload.
• To inform the development of improved management strategies for these hemoglobinopathies.

Main Methods:

• Retrospective analysis of mortality data for 2475 patients with thalassaemia and SCD over a 12-year period in Greece.
• Recording causes of death, comorbidities, treatment details, and iron overload status.
• Statistical comparison of mortality rates and age at death between thalassaemia and SCD cohorts.

Main Results:

• An overall annual mortality rate of 1.13% (335 deaths) was recorded.
• Leading causes of death in thalassaemia were cardiac failure, hepatocellular carcinoma, and sepsis.
• In SCD, sepsis, liver failure, and stroke predominated, with higher sepsis mortality in transfused patients.
• Median age at death was significantly lower for thalassaemia (50 years) than SCD (58.49 years).

Conclusions:

• Significant differences in causes of death and comorbidities necessitate enhanced management strategies for thalassaemia and SCD.
• Optimizing iron overload management, chelation therapy, and hydroxyurea use in SCD can improve survival.
• Targeted interventions and strengthened monitoring protocols are vital for reducing mortality in hemoglobinopathies.