title: Relevant bleeding diathesis due to acquired factor XIII deficiency.
abstract: BACKGROUND
  Acquired factor XIII (FXIII) deficiency is associated with reduced clot firmness and increased bleeding in patients undergoing major surgery. In contrast, only limited information is available on the haemostatic relevance of acquired FXIII deficiency in non-surgical patients.
  
  
  METHODS
  An 81-year-old patient, who had experienced acute type-A dissection of the aorta eight years earlier, presented with a 3-year history of progressive mucocutaneous and soft-tissue bleeding. Diagnostic work-up was unremarkable for global coagulation tests, but FXIII and alpha2-antiplasmin were decreased to 33% and 27%, respectively, while plasma D-dimer was elevated to > 35 mg/l. A FXIII inhibitor was excluded by mixing studies. CT scanning revealed a massively elongated and progressively dilated aorta with a false lumen reaching from the left carotid artery to the iliac bifurcation. Bleeding control was achieved by single doses of FXIII at 20-30 IU/kg body weight and tailored oral tranexamic acid.
  
  
  CONCLUSIONS
  Acquired FXIII deficiency with activity levels of 30-35% may confer a severe bleeding tendency in non-surgical patients, especially in the context of increased thrombin an fibrin generation.
authors.affiliation: Priv.-Doz. Dr. med. Florian Langer, II. Medizinische Klinik und Poliklinik, Hubertus Wald Tumorzentrum - Universitäres Cancer Center Hamburg (UCCH), Universitätsklinikum Eppendorf, Martinistr. 52, 20246 Hamburg, Germany, Tel. +49/(0)40/741 05 24 53, Fax +49/(0)40/741 05 51 93, E-mail: langer@uke.de.
authors.forename: M
authors.initials: M
authors.lastname: Janning
authors.forename: K
authors.initials: K
authors.lastname: Holstein
authors.forename: B
authors.initials: B
authors.lastname: Spath
authors.forename: C
authors.initials: C
authors.lastname: Schnabel
authors.forename: P
authors.initials: P
authors.lastname: Bannas
authors.forename: C
authors.initials: C
authors.lastname: Bokemeyer
authors.forename: F
authors.initials: F
authors.lastname: Langer
mesh_terms: D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005169:Factor VIII / Q000032:analysis*; D005177:Factor XIII Deficiency / Q000097:blood* / Q000150:complications / Q000175:diagnosis*; D006470:Hemorrhage / Q000097:blood* / Q000175:diagnosis* / Q000209:etiology; D006474:Hemorrhagic Disorders / Q000097:blood / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008297:Male
chemical_list: D005169:Factor VIII
keywords: DIC; Faktor XIII; aortic aneurysm; fibrinolysis; tranexamic acid