Relevant bleeding diathesis due to acquired factor XIII deficiency

TY - JOUR

T1 - Relevant bleeding diathesis due to acquired factor XIII deficiency

AU - Janning, M

AU - Holstein, K

AU - Spath, B

AU - Schnabel, C

AU - Bannas, P

AU - Bokemeyer, C

AU - Langer, F

PY - 2013/1/1

Y1 - 2013/1/1

N2 - 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.CASE REPORT: 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. ConcLUSION: 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.

AB - 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.CASE REPORT: 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. ConcLUSION: 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.

KW - Aged, 80 and over

KW - Diagnosis, Differential

KW - Factor VIII

KW - Factor XIII Deficiency

KW - Hemorrhage

KW - Hemorrhagic Disorders

KW - Humans

KW - Male

M3 - SCORING: Journal article

C2 - 24169946

VL - 33 Suppl 1

SP - S50-4

JO - HAMOSTASEOLOGIE

JF - HAMOSTASEOLOGIE

SN - 0720-9355

ER -