Which disorders are included in the differential diagnoses of acquired 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

Acquired disorders

FXIII deficiency should be considered in patients with recurrent miscarriages and recurrent intracranial bleeding.

Any cause of disseminated intravascular coagulation (DIC) can lead to an acquired reduction in fibrinogen and FXIII.

Liver disease may result in decreased production of FXIII and fibrinogen (also can produce dysfibrinogenemia).

Cardiopulmonary bypass: Activation of hemostasis is an integral part of any cardiopulmonary bypass procedure. FXIII antigen levels and clot strength as measured by thromboelastography (TEG) were reduced in parallel with platelet count and fibrinogen levels after bypass; changes were secondary to increased thrombin generation. [85]

Malarial infections: Malaria affects a sizable population worldwide. Patients who are severely ill with falciparum malaria have activity levels of subunit A lower than 50%, with an increase during antiparasitic therapy. An inverse relationship has been found between FXIII levels, clinical severity of the disease, degree of parasitemia, and human neutrophil elastase levels. [86]

A significant reduction in FXIII subunit A levels has been found in patients with active Crohn disease, ulcerative colitis, and infectious colitis, without any change in the levels of the carrier protein (subunit B). A reduction in FXIII activity was associated with a concomitant increase in fibrinogen/fibrin split products correlating with inflammatory bowel disease activity during a yearlong follow-up study of patients with severe ulcerative colitis. The predictive value of such changes is yet to be confirmed in prospective clinical trials.

Reduced levels of FXIII have been reported in patients with scleroderma and Henoch-Schönlein purpura and in advanced malignancies. [87]

Following systemic thrombolytic therapy, severe reduction in the fibrinogen level (hypofibrinogenemia) also is associated with a dysfibrinogenemia. A specimen obtained from a patient who has systemic fibrinolytic effects may show a false-positive urea solubility test result because of an acquired abnormality in the substrate for FXIII (dyshypofibrinogenemia).


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