DEFICIENCY OF ALPHA 2-ANTIPLASMIN

• Biochemical aspects:
  Alpha 2-antiplasmin, a glycoprotein with a molecular weight of about 70,000, is the primary physiologic inhibitor of plasmin. In its absence, plasmin is free to lyse fibrinogen and other plasmin-sensitive proteins.
• Pathological Basis:
  • Mode of inheritance: autosomal recessive (extremely rare, known as Miyasato disease).
  • Patients with hereditary deficiency of alpha 2- antiplasmin have a lifelong history of severe bleeding.
  • Acquired states are seen in: DIC, severe liver disease, septicemia, urokinase or streptokinase therapy, obstructive peripheral vascular disease, primary fibrinolysis (e.g. with neoplastic diseases).
• Treatment:
  • FFP.
  • Adjuvant therapy: Epsilon-aminocaproic acid (EACA) or tranexanic acid.