Probabilities for Congenital Coagulopathies Causing ICHa
| Condition . | Prevalence of Condition, Upper Limits . | Prevalence of ICH, Upper Limits . | Probabilityb . |
|---|---|---|---|
| VWD | 1/1000 | Extremely rare | Low |
| Factor II deficiency | 1/1 million | 11% | 1/10 million |
| Factor V deficiency | 1/1 million | 8% of homozygotes | 1/10 million homozygotes |
| Combined factors V and VIII deficiencies | 1/1 million | 2% | 1/50 million |
| Factor VII deficiency | 1/300 000 | 4%–6.5% | 1/5 million |
| Factor VIII deficiency | 1/5000 males | 5%–12% | 1/50 000 males |
| Factor IX deficiency | 1/20 000 males | 5%–12% | 1/200 000 males |
| Factor X deficiency | 1/1 million | 21% | 1/5 million |
| Factor XI deficiency | 1/100 000 | Extremely rare | Low |
| Factor XIII deficiency | 1/2 million | 33% | 1/6 million |
| α-2 antiplasmin deficiency | 40 cases reported | Not reported | Low |
| Plasminogen activator inhibitor-1 deficiency | Extremely rare | Common | Low |
| Afibrinogenemia | 1/500 000 | 10% | 1/5 million |
| Dysfibrinogenemia | 1/1 million | Single case report | Low |
| Condition . | Prevalence of Condition, Upper Limits . | Prevalence of ICH, Upper Limits . | Probabilityb . |
|---|---|---|---|
| VWD | 1/1000 | Extremely rare | Low |
| Factor II deficiency | 1/1 million | 11% | 1/10 million |
| Factor V deficiency | 1/1 million | 8% of homozygotes | 1/10 million homozygotes |
| Combined factors V and VIII deficiencies | 1/1 million | 2% | 1/50 million |
| Factor VII deficiency | 1/300 000 | 4%–6.5% | 1/5 million |
| Factor VIII deficiency | 1/5000 males | 5%–12% | 1/50 000 males |
| Factor IX deficiency | 1/20 000 males | 5%–12% | 1/200 000 males |
| Factor X deficiency | 1/1 million | 21% | 1/5 million |
| Factor XI deficiency | 1/100 000 | Extremely rare | Low |
| Factor XIII deficiency | 1/2 million | 33% | 1/6 million |
| α-2 antiplasmin deficiency | 40 cases reported | Not reported | Low |
| Plasminogen activator inhibitor-1 deficiency | Extremely rare | Common | Low |
| Afibrinogenemia | 1/500 000 | 10% | 1/5 million |
| Dysfibrinogenemia | 1/1 million | Single case report | Low |
The probability of having a specific bleeding disorder increases in the setting of a family history of that specific named bleeding disorder or if the patient is from an ethnicity in which a specific bleeding disorder is more common (eg, Ashkenazi Jewish people and factor XI deficiency).
“Probability” indicates the probability that an individual in the general population would have the following specific coagulopathy causing an ICH.