Hereditary deficiency of Factor X is rare and clinical bleeding may range from a minor to severe.


Inheritance: autosomal recessive, with consanguinity common in affected families


Clinical features if mild (Factor X levels >= 15%):

(1) bleeding during surgery or major trauma

(2) bleeding during childbirth (if female)


Clinical features if severe:

(1) retroperitoneal hematoma

(2) hemarthroses, which may be crippling

(3) soft tissue hematomas and/or pseudotumors

(4) intracerebral hemorrhage

(5) hematuria

(6) menorrhagia (if female)


Laboratory findings (assuming no factor replacement):

(1) prolonged PT

(2) prolonged aPTT

(3) normal thrombin time

(4) Russell viper venom test may be normal or prolonged

(5) reduced Factor X levels


Differential diagnosis:

(1) other severe hereditary coagulation defects with hemarthroses

(2) acquired Factor X deficiency


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