Acquired factor XIII deficiency is associated with mortality and is not linked to hypocalcemia

Background We have previously shown that factor XIII (FXIII) levels should be above 30% in an acute care setting. The aim of this analysis was to assess morbidity and mortality in a larger sample of patients with acquired FXIII deficiency, and to explore the potential underlying causes of acquired FXIII deficiency. Material and methods We conducted a retrospective analysis of all patients diagnosed with acquired factor XIII deficiency at a large hospital over a period of 24 months (study ID: NCT04416594; http://www.clinicaltrials.gov). We then assessed standardized clinical and outcome data. Results We included 65 patients with FXIII activity below 70%, and a subsequent analysis included a further 104 patients (including those from our previous report). FXIII activity was significantly lower in patients who massively bled and in those admitted to the intensive care unit (ICU). Low FXIII activity was significantly associated with longer ICU stays, a high incidence of major bleeding and multiple organ dysfunction syndrome (MODS), and increased 1- and 2-year mortality. However, we found no evidence of deranged FXIII activation in this acute care setting. Conclusions Acquired factor XIII deficiency is associated with high morbidity and mortality rates. In the setting of acquired FXIII deficiency, the level of FXIII required for haemostasis and endothelium stabilization seem to be around 40%. Activation of FXIII seems to be warranted in order to stop bleeding.