Age

Overall, males with hemophilia in the Registry were slightly younger than those in the HTC PP. Compared to the 2014 U.S. male population,[1] participants in both the Registry and the HTC PP were much younger. Fifty–one percent and 45%, respectively, of the Registry and HTC PP participants with hemophilia were younger than 20 years of age, as compared to only 28% of the U.S. population in this age category. Seventeen percent and 19%, respectively, of Registry and HTC PP participants with hemophilia were 45 years of age or older, compared to 38% of the U.S. population in this age category (Figure 2).

Age Group and Hemophilia Type

Overall, male Registry participants with hemophilia A and hemophilia B were younger than their counterparts in the HTC PP.

Fifty-two percent of male Registry participants with hemophilia A were 20 years of age or younger, compared with 45% in the HTC PP. Sixteen percent of Registry participants with hemophilia A were 45 years of age or older, compared to 18% of those in the HTC PP (Figure 3).

Forty-eight percent of the Registry participants with hemophilia B were 20 years of age or younger, compared with 44% of those in the HTC PP. Twenty-two percent of Registry participants with hemophilia B were 45 years of age or older, compared to 23% of those in the HTC PP (Figure 4).

Figure 2. Age comparison of male Registry participants, male HTC PP participants, and males in the U.S. Census (2014 estimates)
Figure 2. Age comparison of male Registry participants, male HTC PP participants, and males in the U.S. Census (2014 estimates)

Figure 2. Age comparison of male Registry participants, male HTC PP participants, and males in the U.S. Census (2014 estimates)
Age US Census HTC PP Registry
<2 years 4,504,821 2.8% 596 2.9% 370 4.0%
2–10 years 19,850,424 12.5% 4,067 19.7% 2,138 23.3%
11–19 years 19,556,634 12.4% 4,653 22.5% 2,203 24.0%
20–44 years 54,485,511 34.4% 7,413 35.8% 2,919 31.8%
45–64 years 40,641,737 25.7% 2,848 13.8% 1,194 13.0%
65+ years 19,278,040 12.2% 1,107 5.4% 349 3.8%
Figure 3. Age comparison of male Registry and HTC PP participants with hemophilia A
Figure 3. Age comparison of male Registry and HTC PP participants with hemophilia A

Figure 3. Age comparison of male Registry and HTC PP participants with hemophilia A
Age HTC PP Registry
<2 years 446 2.8% 290 4.0%
2–10 years 3,134 19.8% 1,744 24.0%
11–19 years 3,598 22.7% 1,760 24.2%
20–44 years 5,809 36.7% 2,352 32.3%
45–64 years 2,072 13.1% 879 12.1%
65+ years 768 4.9% 251 3.4%
Figure 4. Age comparison of male Registry and HTC PP participants with hemophilia B
Figure 4. Age comparison of male Registry and HTC PP participants with hemophilia B

Figure 4. Age comparison of male Registry and HTC PP participants with hemophilia B
Age HTC PP Registry
<2 years 150 3.1% 80 4.2%
2–10 years 933 19.2% 394 20.8%
11–19 years 1,055 21.7% 443 23.4%
20–44 years 1,604 33.0% 567 29.9%
45–64 years 776 16.0% 315 16.6%
65+ years 339 7.0% 98 5.2%

Severity by Age and Hemophilia Type

Among male participants with hemophilia A, most Registry participants, ages 0–44 years, were categorized as severe and the plurality of participants ages 45–64 years were categorized as severe (44%). However, among Registry participants ages 65 years and older, only 21% were categorized as severe; most (59%) were categorized as mild. Hemophilia A severity in the HTC PP showed a similar pattern by age as was seen in Registry participants (Table 2).

Among male participants with hemophilia B, a different pattern was seen. In the Registry, the majority of male babies and children younger than 2 years of age with hemophilia B were categorized as severe (53%), while in the total HTC PP, the proportion of severe hemophilia B for this age group was lower (36%). In the Registry, the proportion of hemophilia B categorized as severe was 44% among children 2–10 years of age, 38% among adolescents 11–19 years of age, and 43% among adults 20–44 years of age, while in the total HTC PP, the corresponding proportions of severe hemophilia B were lower. In both the Registry and the HTC PP, the proportion of hemophilia B categorized as severe was near 30% among participants between the ages of 45 to 64 and near 20% among participants 65 years of age and older (Table 3).

In both the Registry and the HTC PP, the proportion of hemophilia B categorized as moderate was much higher than the proportion of hemophilia A categorized as moderate in all age groups (Table 3 versus Table 2).

Table 2. Severity level among male Registry and HCT PP participants with hemophilia A, by age
Registry HTC PP
Mild Moderate Severe Mild Moderate Severe
n % n % n % n % n % n %
<2 years 34 11.7% 58 20.0% 195 67.2% 118 26.5% 76 17.0% 238 53.4%
2–10 years 355 20.4% 298 17.1% 1,082 62.0% 856 27.3% 586 18.7% 1,660 53.0%
11–19 years 409 23.2% 292 16.6% 1,053 59.8% 1,085 30.2% 652 18.1% 1,847 51.3%
20–44 years 421 17.9% 383 16.3% 1,532 65.1% 1,373 23.6% 1,029 17.7% 3,359 57.8%
45–64 years 307 34.9% 180 20.5% 382 43.5% 804 38.8% 401 19.4% 847 40.9%
65+ years 147 58.6% 51 20.3% 52 20.7% 456 59.4% 155 20.2% 149 19.4%
Age total 1,673 23.0% 1,262 17.3% 4,296 59.0% 4,692 29.6% 2,899 18.3% 8,100 51.2%

*45 patients in the Registry and 136 patients in HTC PP with unknown severity are not shown

Table 3. Severity level among male Registry and HTC PP participants with hemophilia B, by age
Registry HTC PP
Mild Moderate Severe Mild Moderate Severe
n % n % n % n % n % n %
<2 years 8 10.00% 28 35.00% 42 52.50% 42 28.00% 48 32.00% 54 36.00%
2–10 years 77 19.50% 145 36.80% 172 43.70% 256 27.40% 389 41.70% 274 29.40%
11–19 years 102 23.00% 174 39.30% 167 37.70% 299 28.30% 466 44.20% 285 27.00%
20–44 years 120 21.20% 201 35.40% 243 42.90% 390 24.30% 619 38.60% 581 36.20%
45–64 years 88 27.90% 125 39.70% 97 30.80% 238 30.70% 319 41.10% 212 27.30%
65+ years 38 38.80% 39 39.80% 20 20.40% 140 41.30% 145 42.80% 53 15.60%
Age total 433 22.80% 712 37.50% 741 39.10% 1,365 28.10% 1,986 40.90% 1,459 30.00%

*11 patients in the Registry and 47 patients in HTC PP with unknown severity are not shown