Unravelling the spectrum of von Willebrand factor variants in quantitative von Willebrand disease: results from a German cohort study.

Background: Von Willebrand disease (VWD), the most prevalent hereditary bleeding disorder, results from deficiency of von Willebrand factor (VWF).

Objectives: This large cohort study aims to offer a comprehensive exploration of mutation spectra and laboratory features in quantitative VWF deficiencies, shedding light on genetic underpinnings and genotype-phenotype associations.

Methods: Our cohort consisted of 221 Caucasian index patients with quantitative VWD, along with 47 individuals whose plasma VWF levels fell within the lower normal boundaries (50-70 IU/dL).

Influencing Factors and Differences in Born Aggregometry in Specialized Hemostaseological Centers: Results of a Multicenter Laboratory Comparison.

 Light transmission aggregometry (LTA) is regarded as the gold standard in platelet function diagnostics. However, there is a relevant degree of interlaboratory variability in practical applications.  The aim of the present study was to develop a practicable laboratory comparison on LTA and to analyze differences and influencing factors in regard to standardization in five specialized hemostaseological centers.

Factor-XIII activity in patients with mild to moderate ulcerative colitis and active bleeding: a prospective observational study.

Objective: Coagulation factor XIII plays a key role in fibrin clot stabilization and epithelial healing. Under chronic inflammatory conditions involving bleeding and an activation of the coagulation cascade, the FXIIIa inversely correlate with disease activity. We assumed that FXIIIa could be a predictor of severity in patients with ulcerative colitis (UC).

[Prekallikrein (Fletcher Factor) Deficiency and Prolongation of APTT Reaction].

Background: Prekallikrein (Fletcher factor) is a protein of the contact phase of the blood coagulation system. A deficiency is a very rare event. The clinical meaning is still unknown.