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Relevant bleeding diathesis due to acquired factor XIII deficiency.
M Janning1, K Holstein, B Spath
1Priv.-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,
Hamostaseologie
|October 31, 2013
Summary
Acquired factor XIII (FXIII) deficiency can cause severe bleeding in non-surgical patients. This case highlights the importance of recognizing FXIII deficiency in patients with unexplained bleeding, even without surgery.
Area of Science:
- Hematology
- Coagulation Disorders
Background:
- Acquired factor XIII (FXIII) deficiency is known to impair clot firmness and increase bleeding risk in surgical patients.
- Limited data exist on the hemostatic significance of acquired FXIII deficiency in non-surgical contexts.
Observation:
- An 81-year-old patient with a history of aortic dissection presented with 3 years of progressive mucocutaneous and soft-tissue bleeding.
- Coagulation tests were normal, but FXIII and alpha2-antiplasmin levels were low (33% and 27%), with elevated D-dimer (>35 mg/l).
- Imaging revealed a significantly dilated aorta with an extensive false lumen; FXIII inhibitor was ruled out.
Findings:
- Severe bleeding tendency in non-surgical patients can be associated with acquired FXIII deficiency at activity levels of 30-35%.
- This risk is amplified in the presence of increased thrombin and fibrin generation.
Implications:
- Highlights the potential for significant bleeding complications from acquired FXIII deficiency in non-surgical settings.
- Suggests the need for careful coagulation assessment, including FXIII levels, in non-surgical patients with unexplained bleeding.
- Effective management can be achieved with FXIII concentrate and tranexamic acid.
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