Complications

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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
	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
	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
	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
	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
	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%	*	*