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Elevated urokinase‐type plasminogen activator level and bleeding in amyloidosis: Case report and literature review
Author(s) -
Sane David C.,
Pizzo Salvatore V.,
Greenberg Charles S.
Publication year - 1989
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830310111
Subject(s) - plasminogen activator , amyloidosis , fibrinolysis , urokinase , plasmin , medicine , caproic acid , tissue plasminogen activator , bleeding diathesis , endocrinology , gastroenterology , chemistry , biochemistry , platelet , enzyme , organic chemistry
Hyperfibrinolytic states are reported to be a cause of bleeding in patients with amyloidosis. We reviewed the literature on excessive fibrinolysis in association with amyloidosis and report our findings from a patient with idiopathic amyloidosis who developed a bleeding diathesis. Coagulation laboratory studies indicated elevated plasminogen activator levels associated with a reduction of plasminogen and α 2 ‐plasmin inhibitor (α 2 ‐PI) levels. The level of tissue‐type plasminogen activator (t‐PA) inhibitor and t‐PA antigen were normal. However, the patient did have a five‐ to sevenfold increase in amidolytic activity for the urokinase substrate pyro‐Glu‐Gly‐Arg‐pNA (S‐2444). This case therefore represents a novel example of a hyperfibrinolytic state associated with amyloidosis caused by elevated urokinase‐type plasminogen activator (u‐PA). Epsilon‐amino caproic acid (EACA) therapy resulted in an increase in α 2 ‐PI and plasminogen levels and effectively reduced the blood loss. Hyperfibrinolytic states in amyloidosis have now been reported to be due to elevated t‐PA and u‐PA and depleted t‐PA inhibitor.