Hereditary pseudohaemophilia

  title={Hereditary pseudohaemophilia},
  author={E. von 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… Expand
8 Citations
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. Expand
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. Expand
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. Expand
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. Expand
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. Expand
Optimization of von Willebrand factor multimer analysis in vertical mini-gel electrophoresis systems: A rapid procedure.
An uncomplicated technique to provide agility in the analysis of VWF:MD is developed, which is cheap, rapid, reproducible, easy to be performed, and uses electrophoresis and Western blotting systems available in most laboratories. Expand
European principles of care for women and girls with inherited bleeding disorders
Ten practical principles of care for WGBD evolved from an iterative process among stakeholders, supported by relevant medical societies worldwide, and can serve as a benchmark for diagnosis and comprehensive multidisciplinary management of W GBD, and improve awareness of their unique challenges. Expand