Objective: To investigate the occurrence of ischemic stroke in patients
with congenital bleeding disorders.
Patients and Methods: Patients with congenital bleeding disorders who presented an
objectively proven ischemic stroke were obtained by means of a time unlimited
Pubmed search. Appropriate key words and Medical subject headings were used.
Cross-checking of the references was also carried out.
Results: There were four cases of Fibrinogen defects or Factor VII deficiency; seven
patients had Hemophilia (6 hemophilia A and 1 hemophilia B); eight cases of FXI
deficiency and six patients with von Willebrand Disease. One patient had a
congenital platelet disorder.
Age varied from 7 to 80 (mean age 38.6). 15 were male and 11 female. In four patients gender was not
reported. The ratio of Myocardial Infarction to Ischemic stroke was 12.28 for the hemophilias, 7.83 for
vW Disease and 2.75 for the Rare Bleeding Disorders. Risk factors were present in most patients,
replacement therapy and old age being the most frequent.
Conclusions: The differences in the prevalence of ischemic stroke vs coronary arterial disease seen in
patient with congenital bleeding disorders indicate that clotting defects assure an uneven protection from
atherothrombosis. Since the mechanisms underlying these differences are unknown, they represent a field
of potential future investigation.