Excessive fibrinolysis in amyloidosis associated with elevated plasma single-chain urokinase.
Severe bleeding resulting from excessive fibrinolysis has been observed in patients with primary amyloidosis. The authors studied a patient with this hemostatic disorder before and during therapy with epsilon-aminocaproic acid. Excessive fibrinolysis was associated with depressed plasma concentrations of coagulation Factors XII, XI, high-molecular-weight kininogen, and Factors VIII and V; and plasminogen and alpha-2-plasmin inhibitor. These deficiencies were corrected with treatment. The functional and antigenic concentrations of tissue plasminogen activator and plasminogen activator inhibitor in the patient's plasma were normal. Urokinase-type activator activity and antigen were three to five times elevated in the patient's plasma. Results of immunoprecipitation showed that single-chain urokinase-type activator was the primary urokinase-type activator species in the patient's plasma. Excessive fibrinolysis in patients with amyloidosis results from increased plasma single-chain urokinase-type activator activity.
Liebman
H A
HA
William B. Castle Hematology Research Laboratory, Division of Hematology-Oncology, Boston City Hospital, Massachusetts.
Carfagno
M K
MK
Weitz
I C
IC
Berard
P
P
Diiorio
J M
JM
Vosburgh
E
E
Simms
R W
RW
D000686:Amyloidosis / Q000097:blood / Q000201:enzymology* / Q000503:physiopathology*; D005165:Factor V / Q000032:analysis; D005167:Factor VII / Q000032:analysis; D005172:Factor XI / Q000032:analysis; D005174:Factor XII / Q000032:analysis; D005342:Fibrinolysis / Q000502:physiology*; D006801:Humans; D007704:Kininogens / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010960:Plasminogen Activators / Q000097:blood; D015849:Plasminogen Inactivators / Q000097:blood; D011233:Precipitin Tests; D014568:Urokinase-Type Plasminogen Activator / Q000097:blood*
D007704:Kininogens; D015849:Plasminogen Inactivators; D005165:Factor V; D005167:Factor VII; D005174:Factor XII; D005172:Factor XI; D010960:Plasminogen Activators; D014568:Urokinase-Type Plasminogen Activator