Nonimmune-acquired factor XIII deficiency: a cause of high volume and delayed postoperative hemorrhage

Abstract

Factor XIII (FXIII) levels may decrease because of surgical consumption. Acquired FXIII deficiency could be a cause of postoperative hemorrhage usually underdiagnosed in clinical practice. To determine the diagnosis confirmation rate of acquired FXIII deficiency in postsurgical patients with clinical suspicion and to compare the characteristics and evolution of patients with or without FXIII deficiency. We performed a retrospective cohort study, which included 49 inpatients who were attended at our university hospital from 2014 to 2018 with suspicion of acquired FXIII deficiency because of disproportionate postoperative hemorrhage. FXIIIA levels less than 50% was considered a deficiency. Persistence of bleeding for more than 48 h, drop in hematocrit points, red blood cells transfused units, hemoglobin levels 12–36 h after bleeding, and time elapsed from the procedure to the bleeding were assessed as outcome variables. Logistic regression was employed for both univariate and multivariate analyses. Of the 49 patients included, 27(55%) had FXIII deficiency, with a median level of 34% [interquartile range (IQR) 19–42]. Abdominal surgery was the most common [n = 21 (43%)]. All patients had routine coagulation tests within the hemostatic range. FXIII deficiency was associated with a drop of more than 4 points in hematocrit [OR 59.69 (95% CI 4.71–755.30)], red blood transfused units >2 [OR 45.38 (95% CI 3.48–590.65)], and delayed bleeding >36 h after surgery [OR 100.90 (95% CI 3.78–2695.40)]. Plasma-derived FXIII concentrate was administered to eight patients with life-threatening bleeding with resolution within 24 h. Only one deficient patient died from bleeding. FXIII levels were measured 15 days after diagnosis or more in 20 out of 27 deficient patients, with normal results. Acquired FXIII deficiency may be a frequent underdiagnosed entity that should be considered when high-volume and delayed postoperative hemorrhage is present in patients with hemostatic routine coagulation test results.

Acute Occlusion of the Ventriculoperitoneal Shunt Due to Factor XIII Deficiency-related Postoperative Hemorrhage: A Case Report

“… 16 , 17) Fortunately, in this case, the patient’s consciousness and general condition improved after shunt reconstruction. His F13 level spontaneously increased within a month, which is consistent with the report by Chuliber et al 18) describing that 74% (20 out of 27) of patients with nonimmune-acquired F13 deficiency normalized F13 in 15 days or more after diagnosis. If there is persistent bleeding, persistent decrease in F13, or if F13 deficiency is known or expected preoperatively, administering F13 concentrate should be considered.…”

Management of severe peri-operative bleeding: Guidelines from the European Society of Anaesthesiology and Intensive Care

“…[538][539][540] In surgical patients, FXIII levels less than 60% have been associated with increased postoperative rebleeding and transfusion including cardiac and neurosurgical procedures. [541][542][543][544][545][546][547][548][549] In a double-blinded randomised trial, untargeted administration of FXIII showed no effect, neither on blood transfusion nor on re-operation in cardiac surgery patients. 361 Additionally, FXIII may have some beneficial effects in surgical wound healing and burn injury.…”

The impact of acquired coagulation factor XIII deficiency in traumatic bleeding and wound healing

“…Furthermore, in 49 post-surgical patients with suspected acquired FXIII deficiency, FXIII levels < 50% were identified in 55% of patients. FXIII deficiency was associated with decreased haematocrit, increased transfusion requirement and delayed bleeding [52]. Conversely, treatment with FXIII reduced post-operative blood loss and blood transfusion after coronary surgery in patients where post-medication levels of FXIII ≥ 70% could be achieved [31].…”