1995 年 41 巻 11 号 p. 991-996
Cogenital deficiency of α 2-plasmin inhibitor (α 2-PI) is a very rare hereditary disease associated with poor hemostasis. We successfully performed hemostatic management for intraoral bleeding on 11 occasions in three sisters with this condition in the manner described below.
Hemostatic laboratory tests in these patients showed an accelerated whole blood clot lysis time (3-4 h) and a short euglobulin lysis time (1.5-2 h). A very low activity and concentration of a 2-PI were also characteristically noted.
The following policy is recommended for the management of hemostasis:
1) For surgery, hemostasis can be obtained by oral administration of 10mg/kg of tranexsamic acid (t-AMCHA) every 6 hours (40mg/kg/day) from 3 hours preoperatively to the 7 th postoperative day.
2) When urgent hemostasis is necessitated by continuous bleeding, intravenous injection of 50mg/kg of t-AMCHA followed by oral administration of 25mg/kg/day of t-AMCHA for several days is effective for the maintainance of a high level of serum α 2-PI activity and proper hemostasis.
3) Hemostasis can be achieved by oral administration of 25-35mg/kg/day of t-AMCHA for several days in the case of minor superficial bleeding and hematoma.
4) Proper local hemostatic management is necessary for intraoral bleeding, in addition to improving the abnormal fibrinolytic state by t-AMCHA.