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TABLE 3

Probabilities for Congenital Coagulopathies Causing ICHa

ConditionPrevalence of Condition, Upper LimitsPrevalence of ICH, Upper LimitsProbabilityb
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 
ConditionPrevalence of Condition, Upper LimitsPrevalence of ICH, Upper LimitsProbabilityb
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 
a

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).

b

“Probability” indicates the probability that an individual in the general population would have the following specific coagulopathy causing an ICH.

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