Probabilities for Congenital Coagulopathies Causing ICHa

ConditionPrevalence of Condition, Upper LimitsPrevalence of ICH, Upper LimitsProbabilityb
VWD1/1000Extremely rareLow
Factor II deficiency1/1 million11%1/10 million
Factor V deficiency1/1 million8% of homozygotes1/10 million homozygotes
Combined factors V and VIII deficiencies1/1 million2%1/50 million
Factor VII deficiency1/300 0004%–6.5%1/5 million
Factor VIII deficiency1/5000 males5%–12%1/50 000 males
Factor IX deficiency1/20 000 males5%–12%1/200 000 males
Factor X deficiency1/1 million21%1/5 million
Factor XI deficiency1/100 000Extremely rareLow
Factor XIII deficiency1/2 million33%1/6 million
α-2 antiplasmin deficiency40 cases reportedNot reportedLow
Plasminogen activator inhibitor-1 deficiencyExtremely rareCommonLow
Afibrinogenemia1/500 00010%1/5 million
Dysfibrinogenemia1/1 millionSingle case reportLow
  • 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.