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Severe hemorrhagic tendency in heterozygous alpha 2-antiplasmin deficiency.

作者信息

Kordich L, Feldman L, Porterie P, Lago O

出版信息

Thromb Res. 1985 Dec 1;40(5):645-51. doi: 10.1016/0049-3848(85)90302-0.

DOI:10.1016/0049-3848(85)90302-0
PMID:4089832
Abstract

We report a case of partial deficiency of alpha 2-antiplasmin. A 45 year old Spanish man had a life long severe bleeding tendency after trauma. Routine coagulation and platelet functional tests in symptomatic and asymptomatic periods were normal. During the bleeding there was a rapid whole blood clot lysis and the concentration of alpha 2-antiplasmin in plasma was 20 to 25% of normal controls. After recovery these levels showed a slight increase (35 to 45% of normal). Addition of normal plasma to the patient's plasma increased alpha 2-plasmin inhibitor activity. Bidimensional electrophoresis of alpha 2-antiplasmin in the patient's plasma showed a normal pattern. Family studies showed that one of the proband's two sons had a mild hemorrhagic tendency and 40% of functional and antigenic levels of alpha 2-antiplasmin.

摘要

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Clinical disorders of fibrinolysis: a critical review.
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alpha 2-Antiplasmin Enschede: dysfunctional alpha 2-antiplasmin molecule associated with an autosomal recessive hemorrhagic disorder.
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