Causes of Death and Mortality Trends in Individuals with Thalassemia in the United States, 1999-2020

Figures & data

Table 1 Demographic Characteristics of Thalassemia-Related Death in the United States, 1999–2020

Variable	N (%)	AAMR per 1,000,000 (95% CI)	Percentage Change from 1999–2010 to 2011–2020 (%)
Overall	2797 (100.00)	0.42 (0.40–0.43)	−7.50
Sex
Male	1222 (43.69)	0.43 (0.40–0.45)	−7.32
Female	1575 (56.31)	0.40 (0.38–0.42)	−29.79
Race
Non-Hispanic White	1820 (65.07)	0.32 (0.30–0.33)	−11.76
Non-Hispanic Black	489 (17.48)	0.65 (0.59–0.71)	−16.90
Asian or Pacific Islander	395 (14.12)	1.34 (1.20–1.47)	−21.85
Hispanic	73 (2.61)	0.11 (0.08–0.14)	−16.67
Age
<20	206 (7.38)	0.03 (0.03–0.03)	0
20–39	265 (9.48)	0.04 (0.04–0.04)	−40.00
40–59	340 (12.16)	0.05 (0.05–0.05)	0.00
60–79	727 (26.00)	0.09 (1.77–1.85)	−20.00
≥80	1258 (44.99)	0.17 (0.17–0.17)	+5.88
US Census Region
Northeast	660 (23.60)	0.46 (0.42–0.49)	−6.38
Midwest	541 (19.34)	0.34 (0.31–0.36)	−23.68
South	784 (28.03)	0.31 (0.28–0.33)	−20.00
West	812 (29.03)	0.53 (0.49–0.56)	−4.00
2013 Urbanization
Urban	2435	0.43 (0.41–0.45)	−11.36
Rural	362	0.29 (0.26–0.32)	+6.90

Figure 1 (A) Trend of age-adjusted mortality rate of thalassemia-related death between 1999 to 2020. (B) Overall age-adjusted mortality rate of thalassemia-related death stratified by states.

Table 2 Underlying Causes of Death Among Individuals with Thalassemia in the United States, 1999–2020

Underlying Causes of Death by System	Number of deaths (%)	Most Common Causes within Each System (Number of deaths, %)
Cardiovascular	727 (26.0)	Ischemic heart disease (309, 42.5)
		Hypertensive diseases (83, 11.4)
		Cerebrovascular disease (48, 6.6)
		Aortic valve disease (30, 4.1)
		Cardiomyopathy (22, 3.0)
		Atrial fibrillation and flutter (22, 3.0)
Blood and blood-forming organs	579 (20.7)	Thalassemia (254, 43.9)
		Sickle cell anaemia (37, 6.4)
Neoplasm	499 (17.8)	Lung or Bronchus cancer (90, 18.4)
		Myelodysplastic syndrome (42, 8.41)
		Breast cancer (35, 7.0)
Endocrine	176 (6.3)	Disorders of iron metabolism (65, 36.9)
		Diabetes mellitus (44, 25.0)
Respiratory	173 (6.2)	Chronic obstructive pulmonary disease (76, 43.9)
		Pneumonia (21, 14.7)
Nervous system	141 (5.0)	Alzheimer’s disease (87, 61.7)
		Parkinson’s disease (21, 14.9)
Gastrointestinal	136 (4.9)	Liver cirrhosis (41, 30.1)
Mental	91 (3.3)	Dementia (71, 78.0)
Infection	101 (3.0)	Septicaemia (26, 25.7)
		COVID-19 (17, 16.8)
		Chronic viral hepatitis C (10, 9.9)
Others	169 (6.0)	Unspecified fall (14, 8.3)