Condition | Prevalence of condition, upper limits | Prevalence of ICH, upper limits | Probability* |
Von Willebrand disease | 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 deficiency | 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 |
AP deficiency | 40 cases reported | Not reported | Low |
PAI-1 deficiency | Extremely rare | Common | Low |
Afibrinogenemia | 1/500,000 | 10% | 1/5 million |
Dysfibrinogenemia | 1/1 million | Single case report | Low |
ICH: intracranial hemorrhage.
* "Probability" indicates the probability that an individual in the general population would have the following specific coagulopathy causing an ICH. 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).
¶ Estimated probability of any ICH in children <4 years of age is 1 in 140,000 males with hemophilia A and 1 in 465,000 males with hemophilia B (Anderst JD, Carpenter SL, Presley R, et al. Relevance of Abusive Head Trauma to Intracranial Hemorrhages and Bleeding Disorders. Pediatrics 2018; 141).
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