Excessive fibrinolysis in suspected amyloidosis: demonstration of plasmin-alpha 2-plasmin inhibitor complex and von Willebrand factor fragment in plasma.
We performed a hemostatic evaluation in detail in a patient with suspected amyloidosis who was suffering from several bleeding episodes. He had a shortened euglobulin clot lysis time, decreased alpha 2-plasmin inhibitor (alpha 2-PI), decreased plasminogen, elevated tissue-type plasminogen activator (t-PA), elevated plasmin-alpha 2-PI complex, and decreased ratio of ristocetin cofactor to von Willebrand factor (vWF) antigen. Fibrinogen and fibrin/fibrinogen degradation products levels fluctuated, with abnormal values on several occasions. On crossed immunoelectrophoresis, plasmin-alpha 2-PI complex and vWF fragment were demonstrated in the patient plasma. These abnormal findings and bleeding symptoms improved following the administration of tranexamic acid. Discontinuation of tranexamic acid resulted in deterioration of these parameters. These observations indicate that pathologic fibrinolysis (continuous intravascular plasmin generation) characterized by the consumption of alpha 2-PI and plasminogen, formation of plasmin-alpha 2-PI complex, and fragmentation of vWF contributed to the bleeding in this patient. It is important to recognize excessive fibrinolysis as the underlying cause of bleeding in these patients, since specific treatment with antifibrinolytic agents is effective in controlling the bleeding.
D000686:Amyloidosis / Q000097:blood* / Q000188:drug therapy; D000933:Antifibrinolytic Agents / Q000032:analysis*; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005341:Fibrinolysin / Q000032:analysis*; D005342:Fibrinolysis; D006406:Hematoma / Q000209:etiology; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000032:analysis*; D014148:Tranexamic Acid / Q000627:therapeutic use; D000979:alpha-2-Antiplasmin; D014841:von Willebrand Factor / Q000032:analysis*
D000933:Antifibrinolytic Agents; D005338:Fibrin Fibrinogen Degradation Products; D010446:Peptide Fragments; D000979:alpha-2-Antiplasmin; C037742:plasmin-plasmin inhibitor complex; D014841:von Willebrand Factor; D014148:Tranexamic Acid; D005341:Fibrinolysin