Medication Summary
The goal of thrombasthenia therapy is to compensate (partly) for defective platelet function.
Antifibrinolytic agents
Class Summary
These agents inhibit fibrinolysis via inhibition of plasminogen activator substances and, to a lesser degree, through antiplasmin activity. The thrombus that forms during treatment is not lysed as rapidly. Effectiveness is uncertain.
Aminocaproic acid (Amicar)
Synthetic competitive inhibitor of plasminogen activation. Preparations include 250 mg/mL PO syr, 500-mg and 1000-mg PO tab, and IV susp.
Vasopressin analogs
Class Summary
These agents act like antidiuretic hormone (ADH) to increase factor VIII levels (transiently). They also have direct and local effect on vessel walls that produces increase in platelet adhesion. In part, this local hemostatic action of desmopressin may account for the clinical observation that desmopressin shortens the bleeding time, bleeding episodes, or both.
Desmopressin (DDAVP, Stimate)
Synthetic vasopressin analog used to control severe bleeding.
Clotting factors
Class Summary
Hemostasis is the physiologic response to bleeding. Injury and factors released by platelets initiate the coagulation cascade, which is mediated by blood clotting factors. This results in formation of an insoluble fibrin clot, reinforcing the initial platelet plug.
Coagulation factor VIIa, recombinant (NovoSeven)
Vitamin K–dependent GP that promotes hemostasis by activating extrinsic pathway of coagulation cascade. Data for refractory severe bleeding in the setting of platelet allosensitization are limited. Reports of efficacy in refractory severe bleeding in pregnancy and in the perioperative period. Not approved by the FDA for treatment of GT associated bleeding.
Hemostatic agents, topical
Class Summary
These agents are used as an adjunct to achieve hemostasis.
Gelatin, topical absorbable (Gelfoam, Gelfilm)
Used to provide hemostasis in surgery. Can be used for PO and dental surgery and with topical thrombin to stop epistaxis. Available in sponges, dental packs, and sterile powder.
Thrombin (Thrombin-JMI, Reothrom)
Used as an adjunct to achieve hemostasis. Topical thrombin catalyzes the conversion of fibrinogen to fibrin. Available as powder and lyophilized powder for reconstitution.
