CLASSIC hemophilia is a congenital coagulation defect caused by an absence or low concentration of the plasma protein factor VIII,1,2 which results in impairment of the first phase of coagulation.3-5 (Less common forms are caused by deficiencies in factor IX6,7 and factor XI.8) It is carried on a sex-linked recessive gene which is transmitted to some male offspring by a carrier mother, and it can occasionally be seen in female offspring of a carrier mother and a hemophilic father.
Intracranial hemorrhage is now the leading cause of death in all types of hemophiliacs9,10: Kerr11 reported a mortality of 33% and Silverstein12 a mortality of 71%. Approximately 10% of all hemophiliacs have intracranial hemorrhages, of which 45% are in the subdural and epidural spaces.12 Despite this frequency, only 24 attempts at surgical treatment of intracranial hemorrhage in factor-VIII-deficient hemophiliacs have been reported
Olsen ER. Intracranial Surgery in Hemophiliacs: Report of a Case and Review of the Literature. Arch Neurol. 1969;21(4):401–412. doi:10.1001/archneur.1969.00480160073009
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