ABSTRACT
The mainstay of therapy in patients with congenital hemophilia is factor replacement.
However, the development of inhibitors in these patients is a major complication that
represents an important challenge in hemophilia care. Development of inhibitors complicates
the clinical course of severe hemophilia in up to 30% of patients with hemophilia
A and up to 5% of patients with hemophilia B. Although the main short-term objective
of the treatment of alloantibodies against factors VIIII and IX is to control the
bleeding diathesis, the eradication of the inhibitor is the leading long-term goal.
The management of severe bleeding episodes and the definitive eradication of the autoantibody
are also the two main options of the clinical management of patients with acquired
hemophilia, a rare but life-threatening hemorrhagic condition. The most recent options
available for treating patients with acquired hemophilia and congenital hemophilia
with inhibitors are addressed in this review.
KEYWORDS
Inhibitors - hemophilia - bleeding - immune tolerance - therapy
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Massimo FranchiniM.D.
Immunohematology and Transfusion Center, Department of Pathology and Laboratory Medicine
University Hospital of Parma, Parma, Italy
Email: mfranchini@ao.pr.it
