Diagnosis
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In 2014, the number of persons in the Registry was 3,161. The 2014 Registry participants account for 7.3% of the 43,522 unique participants with bleeding disorders who receive care at a U.S. HTC, as counted in the Community Counts HTC PP1. The distribution of the Registry participants across the United States is shown in Figure 1.The three most prevalent diagnoses were hemophilia A (n=1,855, 59%), hemophilia B (n=485, 15%), and VWD, type 1 (n =301, 10%). Other diagnoses each accounted for 2% or less among total participants in the Registry (Table 1). Approximately 66% of participants have a known family history of a bleeding disorder, 26% do not have a history (8% unknown). The specific genetic mutation causing the participant’s bleeding disorder was known for 32%. Forty-six percent had not had genetic testing or the results of testing did not reveal a specific mutation. For 22%, the history of genetic testing for a specific mutation was unknown (data not shown).
Table 1. The Registry and HTC PP participants by diagnosis, 2014
| The Registry | HTC PP | |||
|---|---|---|---|---|
| Diagnosis | N =
3,161 |
(%) | N =
43,522 |
(%) |
| Alpha-2 antiplasmin deficiency | ≤5 | (<1) | 5 | 0.00% |
| Bernard Soulier syndrome | 6 | (<1) | 46 | 0.10% |
| Blood coagulation disorder with impaired clot retraction time | ≤5 | (<1) | 55 | 0.10% |
| Blood coagulation disorder with prolonged bleeding time | 9 | (<1) | 551 | 1.30% |
| Blood coagulation disorder with prolonged coagulation time | ≤5 | (<1) | 207 | 0.50% |
| Factor I, hereditary | 10 | (<1) | 163 | 0.40% |
| Factor II, hereditary | ≤5 | (<1) | 41 | 0.10% |
| Factor V, hereditary | 6 | (<1) | 296 | 0.70% |
| Factor V-VIII, hereditary | ≤5 | (<1) | 14 | 0.00% |
| Factor VII, hereditary | 36 | (1) | 1167 | 2.70% |
| Factor X, hereditary | 9 | (<1) | 139 | 0.30% |
| Factor XI, hereditary | 22 | (<1) | 745 | 1.70% |
| Factor XIII, hereditary | 14 | (<1) | 153 | 0.40% |
| Glanzmann thrombasthenia | 14 | (<1) | 193 | 0.40% |
| Grey platelet syndrome | ≤5 | (<1) | 7 | 0.00% |
| Hemophilia A | 1855 | (59) | 15474 | 35.60% |
| Hemophilia B | 485 | (15) | 4857 | 11.20% |
| Inherited thrombocytopenia | ≤5 | (<1) | 347 | 0.80% |
| Platelet function disorder, hereditary | 49 | (2) | 1701 | 3.90% |
| Platelet release defect | ≤5 | (<1) | 43 | 0.10% |
| Platelet storage pool disease | 14 | (<1) | 2130 | 4.90% |
| VWD2, type 1 | 301 | (10) | 11702 | 26.90% |
| VWD, type 1C | 6 | (<1) | 48 | 0.10% |
| VWD, type 2, type unknown | 11 | (<1) | 333 | 0.80% |
| VWD, type 2A | 53 | (2) | 673 | 1.50% |
| VWD, type 2B | 23 | (<1) | 402 | 0.90% |
| VWD, type 2M | 50 | (2) | 337 | 0.80% |
| VWD, type 2N | ≤5 | (<1) | 101 | 0.20% |
| VWD, type 3 | 53 | (2) | 359 | 0.80% |
| VWD, other | 6 | (<1) | 108 | 0.20% |
| VWD, unknown | 38 | (1) | 859 | 2.00% |
| More than one of these | 73 | (2) | N/A | |
Includes 42 participants from December 2013
Footnotes
- HTC PP data reported as of June 30, 2016
- VWD= von Willebrand Disease
Pages in this Report
- Highlights & Acknowledgements
- Background
- Methods
- Geographic Distribution of Registry Participants
- Registry Characteristics 2014
- ›Diagnosis
- Sex
- Age
- Race/Ethnicity
- Education
- Weight Status
- Health Insurance Coverage
- Health Insurance Coverage by Age
- Viral and Vaccination Status
- Use of Healthcare Services and Absenteeism
- Chronic Diseases, Conditions, or Surgical Procedures
- Technical Notes
- Participating HTCs