Due nuovi casi di emofilia acquisita A

AUTHORS

ABSTRACT

Two new cases of acquired hemophilia A

Acquired hemophilia A is a rare autoimmune syndrome characterized by the presence of autoantibodies directed to clotting factor VIII. This disorder most commonly occurs in the elderly. Although it may be associated with several underlying pathologies, up to 50% of cases are idiopatic. Typical clinical manifestations are extensive cutaneous purpura and internal hemorrhage. The steps of the diagnostic process include: the presence of a prolonged activated partial thromboplastin time, non corrected by incubation with normal plasma, the absence of lupus anticoagulant, the selective deficiency of factor VIII and, finally, the dosage of the inhibitor anti-factor VIII, using the Bethesda assay or its Nijmegen modification. Here we describe two new cases of acquired hemophilia A. After the diagnosis confirmation, patients were treated with a bypassing agent, the recombinant activated factor VII.
This drug directly activates clotting factor X on the surface of activated platelets, causing the burst of thrombin, which is indispensable for the formation of a stable clot able to stop bleeding.

REFERENCES