What are the possible complications of factor XIII (FXIII) deficiency?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Recurrent CNS bleeding is a major problem requiring prophylactic transfusions. Infections, particularly HIV, AIDS, and chronic hepatitis, can lead to death. Interferon alfa has been used to treat chronic viral hepatitis. Multidrug cocktails are used to treat HIV/AIDS, but protease inhibitors can increase risk of bleeding. Some over-the-counter herbal remedies increase the risk of bleeding.

Viral safety in products derived from plasma is ensured through several techniques, ie, heating, pasteurization, SD treatment, and monoclonal antibody purification. These procedures currently free products from HIV and HCV (lipid-enveloped viruses) but do not solve the problem of transmission of non–lipid-enveloped viruses such as HAV, parvovirus B19, and other transfusion-transmitted viruses (TTV).

Even with recombinant products, a possibility exists of contamination with pathogens previously unknown, including new murine viruses. One report shows the presence of TTV in first-generation recombinant products, due to the use of human serum albumin that is contaminated with TTV. [122] Thus, virus-induced illnesses of concern include hepatitis viruses A-E, GB virus C (or hepatitis G virus), the SEN family of viruses, [121] and human herpesvirus 8, [120] all of which constitute emerging pathogens related to transfusion-transmitted illnesses.

Potential transmission of prions causing Creutzfeldt-Jakob disease (CJD) or its variant form (vCJD) in recipients of blood products was a serious concern early in this century. However, no individual with hemophilia nor any other blood product recipient in the United States is known to have developed CJD. A United Kingdom study found that as of May 2015, no new cases of transfusion-associated vCJD had occurred since 2007 and there was no evidence of transfusion transmission of sporadic CJD. [128] A sensitive and specific blood test for vCJD has been developed and has entered clinical use; it could be used to screen blood supplies. [129]

The presence of inhibitors adds another layer of complexity when alloantibodies develop as a consequence of transfusion of blood products. Spontaneous disappearance is a typical feature of autoantibodies, presumably as a response to removal of the antigenic stimulus. Bleeding associated with inhibitors can be life threatening and requires complex care.

Severe economic and emotional problems occur as a result of the recurrent nature of the bleeding.

Complex psychiatric issues arise in the treatment of patients with HIV/AIDS. [126]

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