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BC: Articoli scritti da G. Castelli

Due nuovi casi di emofilia acquisita A
Two new cases of acquired hemophilia A
<p>Acquired hemophilia A is a rare autoimmune syndrome characterized by&nbsp;the presence of autoantibodies directed to clotting factor VIII. This disorder most commonly occurs in the elderly.&nbsp;Although it may be associated with several underlying pathologies, up to 50% of cases are idiopatic. Typical clinical&nbsp;manifestations are extensive cutaneous purpura and internal hemorrhage. The steps of the diagnostic process include:&nbsp;the presence of a prolonged activated partial thromboplastin time, non corrected by incubation with normal plasma, the&nbsp;absence of lupus anticoagulant, the selective deficiency of factor VIII and, finally, the dosage of the inhibitor anti-factor&nbsp;VIII, using the Bethesda assay or its Nijmegen modification. Here we describe two new cases of acquired hemophilia&nbsp;A. After the diagnosis confirmation, patients were treated with a bypassing agent, the recombinant activated factor VII.<br />This drug directly activates clotting factor X on the surface of activated platelets, causing the burst of thrombin, which&nbsp;is indispensable for the formation of a stable clot able to stop bleeding.</p>
Biochimica Clinica ; 38(6) 651-655
Casi clinici - Case report