Autoimmune Acquired Factor XIII Deficiency: A Case Report

Abstract

Autoimmune acquired factor XIII (FXIII) deficiency is a rare disorder characterized by severe spontaneous hematomas and autoantibodies against FXIII. High mortality rates have been reported (18% within a year of diagnosis). We present a 70-year-old patient with recurrent muscular hematomas. The basic hemostasis study and the coagulation factors were within normal ranges. The aggregation platelet study was also normal and von Willebrand disease was excluded. Bearing in mind the recurrent bleeding history and the described laboratory results, we considered a FXIII deficiency, that was confirmed (FXIII<10%). In addition, we suspected an acquired FXIII deficiency since the patient did not report a personal or family history of bleeding and FXIII gene sequencing study was normal. Non-immune causes were ruled out, and plasma autoantibodies against FXIII were detected. Immunosuppression was rapidly initiated to eradicate inhibitor as was hemostatic treatment to obtain bleeding control. Currently, the patient is asymptomatic, but a low level of FXIII inhibitor remains.

Management of autoimmune factor XIII deficiency in a frail, elderly patient

“…In this second case, immunosuppressive therapy was halted before FXIII activity was improved because of her age and low activity of daily life. Another case report also showed discontinuation of standard aggressive immunotherapy owing to adverse effects in a 70-year-old man [19]. Therefore, after acute bleeding is controlled, if the possibility of life-threatening bleeding (e.g., intracranial, intraabdominal, or intrathoracic bleeding) is appropriately explained and the patient agrees that their activity should be restricted, conservative management alone could be a reasonable option.…”

Systemic lupus erythematosus presenting as acquired factor XIII deficiency and spontaneous subdural haemorrhage

“…This acquired factor XIII deficiency can be classified as immune mediated due to autoimmune diseases and nonimmune mediated including increased consumption or reduced production like recent surgery, liver disease, malignancy, sepsis, or drug induced (Isoniazid, phenytoin, penicillin, ciprofloxacin and procainamide). Rarely it can occur due to inhibitor development against factor XIII in inherited deficiency patients receiving factor replacement therapy and can cause severe bleeding (2). Recently it was found that COVID -19 is also associated with acquired factor XIII deficiency (4).…”