Summary
Thromboses in patients with hereditary bleeding disorders are uncommon. However, in
some cases, the co-existence of prothrombotic risk factors may increase the likelihood
of developing thrombotic complications in such patients. This review summarizes the
cases of thrombosis reported in the literature and analyzes the most important risk
factors for thrombosis in patients with a congenital bleeding tendency. In particular
we focus on central venous catheter (CVC)-associated thrombosis, on the thrombotic
complications of coagulation factor concentrate therapy and on the presence of prothrom-botic
gene mutations. Data were identified by searches of the published literature, including
PubMed, references from reviews and abstracts from the most important meetings on
this topic. In conclusion, there is increasing evidence that thrombotic complications
in patients with hereditary bleeding disorders have a multifactorial pathogenesis,
depending on exogenous (coagulation factor replacement therapy, CVC, HIV infection)
and/or endogenous (prothrombotic gene mutations) risk factors.
Keywords
Bleeding disorders - hemophilia - thrombosis