Complications

Detectable Inhibitor

Inhibitors are antibodies that complicate a bleeding disorder by preventing clotting factor concentrate treatment from working. The majority of male Registry participants had no history of detectable inhibitor titers. Participants with severe hemophilia A were much more likely (25%) to have a history of detectable inhibitors than participants with moderate (10%) or mild (4%) hemophilia A. Likewise, participants with severe hemophilia B were much more likely to have a history of detectable inhibitors (9%) than those with moderate or mild hemophilia B (both 1%).

Figure 23. History of detectable inhibitors among male Registry participants with hemophilia A
Figure 23. History of detectable inhibitors among male Registry participants with hemophilia A

Figure 23. History of detectable inhibitors among male Registry participants with hemophilia A
Mild Moderate Severe
Yes 59 3.5% 132 10.5% 1072 25.0%
No 1439 86.0% 1050 83.2% 3052 71.0%
Unknown 175 10.5% 80 6.3% 172 4.0%
Figure 24. History of detectable inhibitors among male Registry participants with hemophilia B
Figure 24. History of detectable inhibitors among male Registry participants with hemophilia B

Figure 24. History of detectable inhibitors among male Registry participants with hemophilia B
Mild Moderate Severe
Yes * * 6 0.8% 64 8.6%
No 391 90.3% 614 86.3% 627 84.7%
Unknown * * 92 12.9% 50 6.7%

* Counts less than six have been suppressed to protect patient confidentiality

Central Venous Access Device (CVAD)

Male Registry participants with hemophilia A were more likely to have had a CVAD placed than participants with hemophilia B (33% vs. 20%) (Table 16).

Table 16. CVAD placement among male Registry participants with hemophilia A or B
Table 16. CVAD placement among male Registry participants with hemophilia A or B
Hemophilia A Hemophilia B
# % # %
Yes 2,430 33.40% 369 19.50%
No 4,594 63.10% 1,470 77.50%
Unknown 252 3.50% 58 3.10%

Intracranial hemorrhage (ICH)

Approximately 8% of male participants with hemophilia experienced ICH prior to Registry enrollment; it was experienced most often among participants with severe hemophilia. Among male Registry participants with severe hemophilia A, 11% had a history of ICH, compared to 5% with moderate hemophilia and 4% with mild hemophilia. Among male Registry participants with severe hemophilia B, 12% had a history of ICH, compared to 6% with moderate hemophilia and 4% with mild hemophilia.

Figure 25. The proportion of male Registry participants with a history of ICH, by hemophilia type and severity
Figure 25. The proportion of male Registry participants with a history of ICH, by hemophilia type and severity

Figure 25. The proportion of male Registry participants with a history of ICH, by hemophilia type and severity
Mild Moderate Severe
Hemophilia A 59 3.5% 65 5.2% 449 10.5%
Hemophilia B 18 4.2% 39 5.5% 85 11.5%

Joint Bleeds

A history of having ever experienced a joint bleed was most common among male Registry participants with severe hemophilia, regardless of hemophilia type (Figures 26 and 27).

Figure 26. The proportion of male Registry participants with hemophilia A who previously experienced a joint bleed, by severity
Figure 26. The proportion of male Registry participants with hemophilia A who previously experienced a joint bleed, by severity

Figure 26. The proportion of male Registry participants with hemophilia A who previously experienced a joint bleed, by severity
Mild Moderate Severe
Yes 822 49.1% 989 78.4% 3,690 85.9%
No 791 47.3% 253 20.0% 559 13.0%
Unknown 60 3.6% 20 1.6% 47 1.1%
Figure 27. The proportion of male Registry participants with hemophilia B who previously experienced a joint bleed, by severity
Figure 27. The proportion of male Registry participants with hemophilia B who previously experienced a joint bleed, by severity

Figure 27. The proportion of male Registry participants with hemophilia B who previously experienced a joint bleed, by severity
Mild Moderate Severe
Yes 183 42.3% 416 58.4% 609 82.2%
No 243 56.1% 278 39.0% 122 16.5%
Unknown 7 1.6% 18 2.5% 10 1.3%

Joint Procedures

Twenty percent of male Registry participants with hemophilia A reported having a joint procedure in the past, compared to 16% of male participants with hemophilia B. As participants aged, they were more likely to have experienced a joint procedure. Two percent of male participants with hemophilia A, ages 2–10 years, experienced a joint procedure, compared to 8% of participants ages 11–19 years, 32% of participants ages 20–44 years, 45% of participants ages 45–65 years, and 40% of participants ages 65 years and older.  Among male hemophilia B participants, approximately 1% of those ages 2–10 years experienced a joint procedure compared to 5% were  of participants ages 11–19 years, 22% of participants ages 20–44 years 37% of participants ages 45–64 years, and 36% of participants ages 65 years and older (Table 17).

Table 17. History of having undergone at least one joint procedure among male Registry participants, by age and hemophilia type
Table 17. History of having undergone at least one joint procedure among male Registry participants, by age and hemophilia type
<2 years 2–10 years 11–19 years 20–44 years 45–64 years 65+ years
# % # % # % # % # % # %
Hemophilia A
Yes * * 33 1.9% 145 8.2% 760 32.3% 397 45.2% 100 39.8%
No 290 100.0% 1,702 97.6% 1,601 91.0% 1,531 65.1% 454 51.6% 144 57.4%
Unknown * * 9 0.5% 14 0.8% 61 2.6% 28 3.2% 7 2.8%
Hemophilia B
Yes * * * * 24 5.4% 123 21.7% 116 36.8% 35 35.7%
No 80 100.0% 387 98.2% 414 93.5% 432 76.2% 187 59.4% 61 62.2%
Unknown * * * * * * 12 2.1% 12 3.8% * *

* Counts less than six have been suppressed to protect patient confidentiality