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Abstract
Introduction: factor XIII (FXIII) plays a key role in stabilizing the fibrin clot. FXIII deficiency can provoke unexplained spontaneous bleeding or disproportionate postoperative hemorrhage in patients
with normal routine coagulation tests. The acquired FXIII deficiency is usually associated with factor consumption during surgery, altered synthesis or presence of inhibitors.
Materials and methods: retrospective cohort study.
Consecutive patients admitted between January 2014 and November 2017 with clinical suspicion
of acquired FXIII deficiency due to unexplained spontaneous bleeding or disproportionate post-procedure
bleeding (surgery or plasmapheresis). All patients presented classical coagulation tests within hemostatic range. The plasma concentration of subunit A of FXIII (FXIII-A) was determined by an immunoturbidimetric assay. Plasma levels below 50% were considered deficiency. The volume of bleeding was assessed by hematocrit fall, transfused red blood cell units (RBCU) and hemoglobin after bleeding. Clinical evolution was evaluated in terms of persistence of bleeding at 48 hours and death due to bleeding.
Results: 63 patients were included, 23 with spontaneous bleeding and 40 with post-procedure bleeding.
Twenty-five patients (40%) presented FXIII deficiency. FXIII deficiency was associated with a greater fall in hematocrit points [Hematocrit fall > 2 points, OR 12.57 (95% CI 3.17-49.7), p = 0.009] and RBCU transfused [> = 1, OR 9 (95% CI 2.56-31.60), p = 0.001]. The acquired FXIII deficiency was associated with delayed bleeding in cases secondary to surgery or plasmapheresis [bleeding time > 48 hours, OR 2.93 (95% CI 1.66-5.18), p =0.001], and the presence of bleeding post procedure [OR 5.25 (95% CI 1.5-18.2, p = 0.009].
Conclusions: acquired FXIII deficiency may be an underdiagnosed cause of post-procedure delayed bleeding. It was associated with greater bleeding volume. Classical coagulation test are usually within the hemostatic range so, a high index of clinical suspicion is needed to request FXIII measurement and thus reach diagnosis.
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