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  2. Alpha-2-plasmin inhibitor (a2-PI) deficiency

Alpha-2-plasmin inhibitor (a2-PI) deficiency

Definition:

Alpha-2-plasmin inhibitor (a2-PI) deficiency is an autosomal recessive disorder resulting in severe hemorrhagic diathesis. Mutations in SERPINF2 gene cause a2-PI deficiency. A2-PI acts as the primary inhibitor of plasminogen, and its deficiency causes a rare bleeding disorder because of increased fibrinolysis.

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