Abstract

Congenital dysfibrinogenemia is a rare autosomal recessive bleeding disorder, which is characterized by the absence of functional fibrinogen. Patients may have bleeding and paradoxical arterial and venous thrombotic problems from early childhood. The optimal antithrombotic therapy in these patients hasn't been determined yet. In this report we present a dysfibrogenemic patient, who has suffered recurrent arterial thrombosis under aspirin treatment. Intravenous fibrinogen concentrates (fc) along with reduced doses of rivaroxaban (10mg daily), cilostazol (50mg bid) and aspirin (100mg daily) were given as antithrombotic treatment. The pain and the cyanosis clinically recovered within 6weeks. This is, to our knowledge, the first time in which a new oral anticoagulant, rivaroxaban and cilostazol combination was used in a dysfibrinogenemic patient with thrombotic episodes. We determined the type, the dosage and the duration of antithrombotic treatment according to the clinical progress of the symptoms. Rivaroxaban, cilostazol and fibrinogen concentrate replacement; combination may represent a useful alternative for the antithrombotic treatment in dysfibrinogenemic patients.

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