What is the role of prophylactic factor replacement 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

To date, prophylactic factor replacement has been undertaken mainly in patients with intracranial bleeding or recurrent miscarriages caused by severe FXIII deficiency. Successful prevention of recurrent joint bleeds also has been accomplished using periodic transfusions of FFP and cryoprecipitate. [3] FFP can be administered in a dose of 2-3 mL/kg every 4 weeks.

A literature review of bleeding risks and reproduction among patients with severe FXIII deficiency suggests that patients with clinically significant bleeding should start receiving factor replacement therapy in childhood to reduce early mortality from hemorrhages and to allow patients to reach adulthood. During pregnancy, monthly replacement was found to be effective in preventing miscarriages. [113] However, both short-term benefits and potential long-term adverse consequences of prophylactic use of these products must be discussed, with full patient participation in all decision making.


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