What is the role of antifibrinolytic agents in the treatment of factor XIII (FXIII) deficiency?

Updated: Apr 02, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Antifibrinolytic agents are not used commonly to treat patients with FXIII deficiency but may be used as ancillary therapy. The hemostatic plug formed in the presence of adequate levels of FXIII at the time of surgical trauma (as with dental procedures or with mucosal bleeding) can be preserved by inhibiting fibrinolysis with ε-aminocaproic acid (EACA; Amicar) or trans-p- aminomethyl-cyclohexane carboxylic acid (AMCA; also termed tranexamic acid; Cyklokapron) administered orally or, if needed, intravenously.

EACA has been administered in a dose of 5 g orally or intravenously slowly prior to the surgical procedure, along with a dose of the appropriate FXIII replacement. This is followed by a maintenance dose of 1 g/h postoperatively until it is appropriate to start tapering the dose over the next several days.

AMCA is administered in a dose of 1.5 g intravenously every 6-8 hours and tapered, as needed. Hhowever, it is not available in the United States.

Antifibrinolytic agents also can be used as a mouthwash for oral bleeding and have been used to stop local intracavitary oozing.

Antifibrinolytic agents are contraindicated in patients with hematuria originating from above the bladder, because of the possible risk of development of a firm occluding clot in the ureters when administered simultaneously with factor replacement (however, many urologists routinely use EACA irrigations after prostatic and bladder surgery)

Antifibrinolytic agents are not useful in the treatment of joint bleeding (see Hemophilia A for more information).


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