How is acquired 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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Patients with acquired inhibitors to FXIII should be treated using well-established principles of therapy. Note the following:

  • FXIII dose depends on the characteristics of the inhibitor. One patient was treated preoperatively using a 10-fold dose of FXIII concentrate (350 U/kg) followed by a similar postoperative dose resulting in adequate hemostasis after coronary bypass graft surgery. [108]

  • In addition to administering an FXIII concentrate whenever available, treat the underlying disorder and, when appropriate, use immunosuppressive agents, including the newer B-cell-directed monoclonal antibodies.

  • Note that spontaneous disappearance of acquired inhibitors is part of their natural history, and the use of milder less toxic immunomodulators, such as steroids, may suffice.

  • The proper choice of agent is dictated by clinical circumstances. Simple immediate ancillary measures of ice, pressure, ace wrap, immobilization of the affected joint, and avoidance of NSAIDs must not be forgotten.

  • The complexity of required treatment is exemplified by a patient with an INH-induced inhibitor in whom INH was discontinued, cryoprecipitate and FXIII concentrate were administered, the patient underwent plasma exchanges and treatment with an immunoadsorption column to reduce the inhibitor's titer, and immunosuppressives were administered before hemostatic success was achieved. [91, 89, 93]

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