Prevalence of Congenital Hemolytic Disorders in Denmark, 2000–2016

Figures & data

Table 1 Characteristics of Patients with Congenital Hemolytic Disorders in Denmark, 2000–2016

	Alpha Thalassemia			Beta Thalassemia				Other Defined Congenital
	Alpha Thalassemia Trait, n=1477	HbH Disease, n=52	Alpha Thalassemia NOS, n=70	Beta Thalassemia Minor, n=2566	Beta Thalassemia Intermedia, n=31	Beta Thalassemia Major, n=52	Thalassemia NOS, n=267	Sickle Cell Trait, n=635	Sickle Cell Disease, n=236	Hereditary Spherocytosis, n=1289
Women (% [95% CI])	74.5 [72.2; 76.7]	57.7 [43.2; 71.3]	61.4 [49.0; 72.8]	58.7 [56.7; 60.6]	61.3 [42.2; 78.2]	67.3 [52.9; 79.7]	58.4 [52.3; 64.4]	68.7 [64.9; 72.3]	55.9 [49.3; 62.4]	48.4 [45.6; 51.2]
Danish origin (% [95% CI])	10.8 [9.3; 12.5]	9.6 [3.2; 21.0]	28.6 [18.4; 40.6]	16.3 [14.9; 17.8]	9.7 [2.0; 25.8]	5.8 [1.2; 15.9]	28.5 [23.1; 34.3]	12.4 [10.0; 15.3]	16.1 [11.7; 21.4]	94.5 [93.1; 95.7]
Splenectomized (% [95% CI])	0.1 [0.0; 0.5]	0.0 [0.0; 6.8]	1.4 [0.0; 7.7]	0.4 [0.2; 0.7]	3.2 [0.1; 16.7]	5.8 [1.2; 15.9]	0.4 [0.0; 2.1]	0.2 [0.0; 0.9]	2.1 [0.7; 4.9]	33.6 [31.0; 36.2]
Age at diagnosis (median (IQR))	28.9 (19.2; 36.9)	27.9 (10.5; 39.2)	16.7 (4.1; 39.2)	28.3 (15.1; 40.2)	18.5 (5.0; 26.1)	17.5 (6.4; 26.3)	23.0 (4.7; 47.6)	32.0 (23.3; 40.6)	17.1 (3.5; 34.4)	8.7 (0.3; 37.4)

Notes: Basic characteristics of patients with congenital hemolytic disorders in Denmark, 2000–2016. Danish origin is all people where at least one of the parents has Danish citizenship.

Abbreviations: CI, confidence interval; HbH, hemoglobin H; IQR, interquartile range; NOS, not otherwise specified.

Table 2 Prevalence of Congenital Hemolytic Disorders in Denmark in 2000, 2007 and 2015

Prevalence per 100,000 Persons [95% CI]
		2000	2007	2015
Alpha Thalassemia
Alpha Thalassemia Trait
	All	0.47 [0.30; 0.69]	6.76 [6.08; 7.48]	19.22 [18.10; 20.40]
	Age <20	0.55 [0.22; 1.14]	7.85 [6.42; 9.51]	22.58 [20.08; 25.29]
	Age 20–50	0.76 [0.45; 1.21]	10.84 [9.53; 12.29]	31.31 [29.06; 33.69]
	Age >50	0.00 [0.00; 0.22]	0.97 [0.58; 1.54]	3.60 [2.83; 4.52]
HbH Disease
	All	0.06 [0.01; 0.16]	0.28 [0.15; 0.45]	0.74 [0.53; 1.00]
	Age <20	0.00 [0.00; 0.29]	0.30 [0.08; 0.77]	1.06 [0.58; 1.79]
	Age 20–50	0.13 [0.03; 0.37]	0.44 [0.21; 0.81]	1.09 [0.71; 1.61]
	Age >50	0.00 [0.00; 0.22]	0.05 [0.00; 0.30]	0.15 [0.03; 0.43]
Alpha Thalassemia NOS
	All	0.21 [0.10; 0.37]	0.42 [0.27; 0.63]	0.90 [0.67; 1.18]
	Age < 20	0.32 [0.09; 0.81]	1.05 [0.57; 1.76]	2.51 [1.73; 3.52]
	Age 20–50	0.21 [0.07; 0.50]	0.27 [0.10; 0.58]	0.70 [0.40; 1.13]
	Age > 50	0.12 [0.01; 0.42]	0.16 [0.03; 0.47]	0.10 [0.01; 0.35]
Beta Thalassemia
Beta Thalassemia Minor
	All	4.45 [3.90; 5.05]	15.44 [14.41; 16.52]	34.91 [33.39; 36.49]
	Age <20	7.13 [5.73; 8.76]	24.91 [22.30; 27.73]	51.77 [47.95; 55.80]
	Age 20–50	5.44 [4.53; 6.46]	19.12 [17.36; 21.01]	47.65 [44.86; 50.56]
	Age >50	1.11 [0.67; 1.73]	4.11 [3.24; 5.14]	9.93 [8.61; 11.39]
Beta Thalassemia Intermedia
	All	0.02 [0.00; 0.10]	0.09 [0.03; 0.21]	0.44 [0.29; 0.65]
	Age <20	0.08 [0.00; 0.44]	0.37 [0.12; 0.87]	1.06 [0.58; 1.79]
	Age 20–50	0.00 [0.00; 0.16]	0.00 [0.00; 0.16]	0.44 [0.21; 0.80]
	Age >50	0.00 [0.00; 0.22]	0.00 [0.00; 0.20]	0.05 [0.00; 0.27]
Beta Thalassemia Major
	All	0.02 [0.00; 0.10]	0.31 [0.18; 0.50]	0.69 [0.49; 0.94]
	Age <20	0.08 [0.00; 0.44]	0.97 [0.52; 1.66]	1.90 [1.23; 2.81]
	Age 20–50	0.00 [0.00; 0.16]	0.18 [0.05; 0.45]	0.57 [0.30; 0.97]
	Age >50	0.00 [0.00; 0.22]	0.00 [0.00; 0.20]	0.05 [0.00; 0.27]
Thalassemia NOS
	All	1.56 [1.24; 1.93]	2.44 [2.04; 2.89]	3.29 [2.83; 3.79]
	Age <20	3.41 [2.46; 4.59]	5.39 [4.21; 6.78]	7.75 [6.32; 9.41]
	Age 20–50	1.32 [0.89; 1.87]	2.12 [1.57; 2.82]	2.84 [2.19; 3.62]
	Age >50	0.53 [0.24; 1.00]	0.70 [0.37; 1.20]	0.92 [0.56; 1.44]
Other Defined Congenital
Sickle Cell Trait
	All	0.71 [0.50; 0.98]	2.13 [1.76; 2.55]	8.11 [7.38; 8.89]
	Age <20	0.79 [0.38; 1.46]	2.24 [1.51; 3.20]	6.92 [5.57; 8.49]
	Age 20–50	1.19 [0.79; 1.72]	3.32 [2.61; 4.16]	13.67 [12.20; 15.27]
	Age >50	0.00 [0.00; 0.22]	0.59 [0.30; 1.06]	2.68 [2.02; 3.48]
Sickle Cell Disease
	All	0.53 [0.35; 0.76]	1.16 [0.89; 1.48]	2.70 [2.29; 3.17]
	Age <20	0.95 [0.49; 1.66]	2.24 [1.51; 3.20]	7.07 [5.71; 8.66]
	Age 20–50	0.42 [0.20; 0.78]	1.24 [0.82; 1.79]	2.18 [1.62; 2.88]
	Age >50	0.35 [0.13; 0.76]	0.27 [0.09; 0.63]	0.49 [0.23; 0.90]
Hereditary Spherocytosis
	All	10.24 [9.40; 11.14]	13.77 [12.80; 14.79]	17.72 [16.64; 18.85]
	Age <20	30.57 [27.60; 33.78]	39.19 [35.91; 42.70]	55.03 [51.10; 59.19]
	Age 20–50	4.71 [3.88; 5.68]	7.12 [6.07; 8.31]	9.04 [7.85; 10.36]
	Age >50	2.86 [2.12; 3.78]	3.51 [2.71; 4.48]	3.50 [2.74; 4.41]

Notes: Prevalences estimated as the number of living persons assigned the diagnosis at the latest on 1 January each of the years 2000, 2007 and 2015 stratified by age and sex with population denominators derived from census data.

Abbreviations: CI, confidence interval; HbH, hemoglobin H; NOS, not otherwise specified.

Figure 1 Prevalence of alpha thalassemic disorders in Denmark, 2000–2016, according to models for classification of diagnoses (main model vs sensitivity model). The overall prevalence with 95% confidence intervals for alpha thalassemic diseases calculated on 1 January each year with census data as denominator. The 95% confidence intervals are calculated using the Clopper–Pearson method. Alpha-Thalassemia trait and HbH disease w not defined in the sensitivity model they do not have a separate ICD 8 or 10 code.

Abbreviations: CI, confidence interval; HbH, hemoglobin H; NOS, not otherwise specified.

Figure 2 Prevalence of beta thalassemic disorders in Denmark, 2000–2016, according to models for classification of diagnoses (main model vs sensitivity model). The overall prevalence with 95% confidence intervals for beta-thalassemic diseases calculated on 1 January each year with census data as denominator. The 95% confidence intervals are calculated using the Clopper–Pearson method.

Abbreviations: CI, confidence interval; NOS, not otherwise specified.

Figure 3 Prevalence of sickle cell disorders and hereditary spherocytosis in Denmark, 2000–2016, according to models for classification of diagnoses (main model vs sensitivity model). The overall prevalence with 95% confidence intervals for sickle cell disorders and hereditary spherocytosis calculated on 1 January each year with census data as denominator. The 95% confidence intervals are calculated using the Clopper–Pearson method.

Abbreviation: CI, confidence interval.