Hereditary pseudohaemophilia

  title={Hereditary pseudohaemophilia},
  author={E. Willebrand},
E. Frank (Breslau), in his recent big treatise on the haemorrhagic diatheses, puts forward that classical haemophilia is such a purely hereditary-familial anomaly that one has to put in question whether sporadic cases of this disease may exist at all. On the other hand, he says, classical thrombopenia is so exclusively sporadic that it may put in question whether familial forms of this disease exist at all. Thrombopenia in this connection is the disease that has in the past carried the name… 

von Willebrand factor alloantibodies in type 3 von Willebrand disease.

The case of a 6-year-old girl with type 3 von Willebrand disease in whom inhibitors were sought due to ineffective haemostasis together with lower than expected von Wilrebrand factor (VWF) recoveries after a surgical procedure successfully stopped the bleeding.

Current Challenges in the Peripartum Management of Women with von Willebrand Disease.

The evidence base to date is reviewed, the current clinical challenges in the management of pregnant women with von Willebrand disease are explored, and standardized methods of quantification of blood loss at the time of delivery are currently lacking.

Towards novel treatment options in von Willebrand disease

Two of such options are discussed in more detail: emicizumab to correct for the deficiency of factor VIII (FVIII), and the pegylated aptamer BT200 to increase endogenous levels of the VWF/FV III complex.

von Willebrand factor/ADAMTS‐13 interactions at birth: implications for thrombosis in the neonatal period

The historical evolution of the methodology used to measure VWF/ADAMTS‐13 and how it may influence the results obtained during the first days of life are described and brought attention to the potential pathophysiologic role of Volkswagen Willebrand factor and its cleaving protease in neonatal thrombosis.

ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease.

Key recommendations of these guidelines include the role of bleeding-assessment tools in the assessment of patients suspected of von Willebrand disease, diagnostic assays and laboratory cutoffs for type 1 and type 2 VWD, how to approach a type 1 VWD patient with normalized levels over time, and therole of genetic testing vs phenotypic assays for types 2B and 2N.

Bleeding Symptoms and von Willebrand Factor Levels: 30-Year Experience in a Tertiary Care Center

Bleeding symptoms were indistinguishable between type 1 VWD and low VWF using the 30 IU/dL cutoff point, however, VWF ristocetin cofactor activity or gain-of-function mutant glycoprotein Ib binding activity and VWF collagen binding activity could predict increased bleeding risk by 92.3% specificity and 70.0% sensitivity.

Age-Associated Increase in Thrombogenicity and Its Correlation with von Willebrand Factor

This review has aimed to discuss the age-related increase in VWF, its potential mechanisms, and associated coagulopathies as probable consequences.

Closing the gap – detection of clinically relevant von Willebrand disease in emergency settings through an improved algorithm based on rotational Thromboelastometry

This new modification of the standard ROTEM assay enables the detection of otherwise unnoticed critical von Willebrand disease based on alterations in clot formation and might serve as a novel approach to reliably assess severe VWD patients by platelet-mediated blood clotting in an emergency setting.

Von Willebrand factor contribution to pathophysiology outside of von Willebrand disease

Assessment of contribution of elevated levels of VWF to various thrombotic and nonthrombosis pathological conditions in patients with von Willebrand disease and related conditions is discussed.