Survival and causes of death in patients with alpha and beta-thalassemia in Northern Thailand

Abstract

Background

Thalassemia is the most prevalent hereditary anaemia worldwide. Severe forms of thalassemia can lead to reduced life expectancy due to disease-related complications.

Objectives

To investigate the survival of thalassemia patients across varying disease severity, causes of death and related clinical factors.

Patients and methods

We conducted a retrospective review of thalassemia patients who received medical care at Chiang Mai University Hospital. The analysis focused on survival outcomes, and potential associations between clinical factors and patient survival.

Results

A total of 789 patients were included in our study cohort. Among them, 38.1% had Hb H disease, 35.4% had Hb E/beta-thalassemia and 26.5% had beta-thalassemia major. Half of the patients (50.1%) required regular transfusions. Sixty-five patients (8.2%) had deceased. The predominant causes of mortality were infection-related (36.9%) and cardiac complications (27.7%). Transfusion-dependent thalassemia (TDT) (adjusted HR 3.68, 95% CI 1.39–9.72, p = 0.008) and a mean serum ferritin level ≥3000 ng/mL (adjusted HR 4.18, 95% CI 2.20–7.92, p < 0.001) were independently associated with poorer survival.

Conclusions

Our study highlights the primary contributors to mortality in patients with thalassemia as infection-related issues and cardiac complications. It also underscores the significant impact of TDT and elevated serum ferritin levels on the survival of thalassemia patients.

Keywords:

• Cardiac complications
• infection
• iron overload
• mortality
• thalassemia
• survival

Acknowledgments

We sincerely thank Ms. Antika Wongthanee, Former Head of the Analytical & Statistical Data Unit, Research Institute for Health Sciences, Chiang Mai University for her invaluable suggestions, and insight in the evaluation of the statistical data obtained in this study.

Disclosure statement

The authors report no conflicts of interest.

Author contributions statement

AT, TK, CC, LR and PC conceived and designed the study. TK and KF collected the data. AT, TK, PP, TR, SH, ER, KF and PC analyzed the data and interpreted the results. AT and TK drafted the manuscript. All revised the manuscript critically for intellectual content. All authors were involved in the final revision of the manuscript, gave contribution to the final analysis of the data and final approval. All authors agree to be accountable for all aspects of the work.

Data availability statement

The medical records of the patients used in this analysis contain identifiable human subject data, which cannot be disseminated under the terms of the IRB and data use agreements with contributing institutions. For analyses of patient-level identifiable data within our trusted research environment, please email the corresponding author.

Additional information

Funding

No funding was received.