Diagnosis & Severity

There are 9,173 male participants in the Registry with a hemophilia diagnosis: 7,276 with hemophilia A and 1,897 with hemophilia B. The Registry participants with hemophilia account for 44% of the unique male participants with a hemophilia diagnosis who receive care at a U.S. HTC, as counted in the Community Counts HTC PP.

Most of the males with hemophilia A, both in the Registry and in the total HTC PP, had severe hemophilia: 59% and 51%, respectively. Seventeen percent of males with hemophilia A in the Registry had moderate hemophilia, compared to 18% of those in the HTC PP. Twenty-three percent of males with hemophilia A in the Registry had mild hemophilia, compared to 30% of those in the HTC PP. This includes 0.6% from the Registry and 1% from the HTC PP who had a factor activity level ≥40%. Less than 1% of males with hemophilia A, enrolled in both the Registry and HTC PP, had an unknown severity level (Table 1).

Among males with hemophilia B, 39% in the Registry had severe hemophilia, compared with 30% of those in HTC PP, and 38% in the Registry had moderate hemophilia B, compared with 41% in the HTC PP. Twenty-three percent of males with hemophilia B in the Registry had mild hemophilia, compared with 28% of those in the HTC PP; these figures include 0.4% from the Registry and 1% from the HTC PP who had a factor activity level ≥40%. One percent of hemophilia B participants in the HTC PP had an unknown severity, compared with 0.6% in the Registry (Table 1).

Table 1. Male Registry and HTC PP participants with hemophilia, by diagnosis and severity

The Registry and HTC PP participants by diagnosis, 2014.
Hemophilia A Hemophilia B
HTC PP The Registry HTC PP The Registry
n % n % n % n %
Mild 4,692 29.6% 1,073 23.0% 1,365 28.1% 433 22.8%
Moderate 2,899 18.3% 1,262 17.3% 1,986 40.9% 712 37.5%
Severe 8,100 51.2% 4,296 59.0% 1,459 30.0% 741 39.1%
Unknown 136 0.9% 45 0.6% 47 1.0% 11 0.6%