A bleeding disorder due to deficiency of alpha 2-antiplasmin.
The investigation of a 33 year old man with a lifelong bleeding tendency is described. Defective fibrinolysis was suspected in 1968, when clinical bleeding was corrected by administration of aminocaproic acid. The paper establishes the diagnosis as alpha 2-antiplasmin deficiency and describes its management with oral tranexamic acid.
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MIYAUCHI, Y., MII, Y., AOKI, M., TAMAI, S., TAKAHASHI, Y., YOSHIOKA, A.
(1996). Operative Treatment of Intramedullary Hematoma Associated with Congenital Deficiency of {{alpha}}2-Plasmin Inhibitor. A Report of Three Cases. JBJS
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Holmes, W., Lijnen, H., Nelles, L, Kluft, C, Nieuwenhuis, H., Rijken, D., Collen, D
(1987). Alpha 2-antiplasmin Enschede: alanine insertion and abolition of plasmin inhibitory activity. Science
238: 209-211
[Abstract]
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