How is factor XIII (FXIII) deficiency treated?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Factor XIII (FXIII) replacement is used to treat bleeding, to prevent perioperative bleeding during elective surgical procedures or, prophylactically, to prevent recurrent bleeding, as in central nervous system (CNS) or joint hemorrhages. Serial monitoring of achieved FXIII levels is essential to document the adequacy of any therapy.

Prompt and adequate therapy for acute bleeding is essential along with immobilization of the affected sites and pain relief. Most patients receive fresh frozen plasma (FFP) or cryoprecipitate to treat bleeding. Information regarding the amount of FXIII present in either of these products usually is not available; therefore, monitoring the adequacy of FXIII levels is essential.

Virus-inactivated FXIII concentrates made from human plasma or placenta are an improvement over traditional products. Human factor XIII concentrate (Corifact) is approved for prophylaxis in congenital FXIII deficiency by the US Food and Drug Administration (FDA). It is marketed under the brand name Fibrogammin P in Europe, South America, South Africa, and Japan. A second FXIII concentrate (Bio Products Laboratory, Elstree, Hertfordshire, UK) is available on a per-patient request.

Factor XIII A-subunit, recombinant (Tretten) was approved by the FDA in December 2013. [102] Approval was based on results from a clinical study that demonstrated the safety and efficacy of rFXIII A-subunit. The phase 3 trial included 41 patients and showed that preventive treatment with monthly 35 IU/kg rFXIII A-subunit injections significantly decreased the number of treatment-requiring bleeding episodes, compared with an historic control group of individuals who did not receive routine FXIII infusions, . [103]

The long half-life of FXIII of 6-19 days and the hemostatic efficacy of even small amounts of FXIII of approximately 5% allow replacement therapy to be administered every 4-6 weeks. An FFP dose of 2-3 mL/kg may be effective for up to 4 weeks. [104, 105] The dose of concentrate in adults with deficiency is 35 U/kg every 4 weeks. [106, 107, 108]

A paucity of data exists concerning the pediatric population. Hemostatic evaluation following a head trauma-induced large subcutaneous hematoma associated with recurrent postsurgical bleeding led to a diagnosis of severe FXIII deficiency in a 22-month-old boy. Following initial therapy, subsequent replacement with an FXIII concentrate dose of 50 U/kg every 5 weeks was sufficient to prevent rebleeding and allow healing. [109] Serial monitoring of actual levels achieved is important in children to determine adequacy of any therapy. For more information, see Pediatric Factor XIII Deficiency.

Minor bleeding, as from cuts and abrasions, may respond to conservative measures, such as pressure, ice, and use of antifibrinolytic drugs. Avoidance of trauma and nonsteroidal anti-inflammatory drugs (NSAIDs) is helpful in reducing bleeding events.

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