Management of VWD

Abstract

VWD is the most common inherited bleeding disorder known. It is caused by a deficiency or dysfunction of the VWF molecule. Bleeding risk varies between modest increases in bleeding seen only with procedures to major risk of spontaneous hemorrhage depending upon the type of VWD. The treatment approach to VWD has changed little in the past 2 decades, but there are numerous subtleties in optimal management. Management includes the prevention or treatment of bleeding by raising endogenous VWF levels with medications such as desmopressin or providing exogenous VWF concentrates. Fibrinolytic inhibitors and topical hemostatic agents are also effective adjunctive measures. Bleeding specific to women presents a special challenge because of heavy menstrual bleeding and pregnancy. Successful management of pregnancy in patients with VWD involves coordination with obstetrics, anesthesia, and the coagulation laboratory monitoring VWF:RCo and FVIII:C levels. Prophylactic treatment with VWF concentrates is emerging as an effective preventive therapy in patients with severe disease. Antibodies to VWF present a special challenge in the management of rare patients with type 3 disease. New therapies on the horizon include recombinant VWF, anti-VWF aptamers, and medications such as IL-11 to raise VWF levels. The key to effective treatment of VWD is an accurate diagnosis of the specific type and selection of hemostatic products appropriate for the clinical situation. Learning Objective• To learn the basic principles of management for all types of inherited VWD to implement into clinical practice

Emergent Self-Organized Criticality in Gene Expression Dynamics: Temporal Development of Global Phase Transition Revealed in a Cancer Cell Line

“…In the plane of expression change versus expression, Fig 2D shows a sharp transition similar to the sandpile model [20,21] as a mean field behavior, where the singular point exists near zero expression change. It is worth reminding that a sandpile is the first and most common model of self-organized-criticality.…”

“…The scaling-divergent profile in DEABs of the expression strongly resembles avalanche size distribution in the sandpile model [21], which sheds light on SOC in the global genetic response as a route for a spatio-temporal genomic phase transition.…”

Thromboelastometry analysis of thrombocytopenic dengue patients: a cross-sectional study

“…Desmopressin is a hormone that stimulates release of Von Willebrand factor (vWF) by endothelial cells [10, 22]. The complex factor VIII (FVIII) and vWF improve platelets aggregation and clot stability [33]. Increased levels of vWF were demonstrated in DVI patients during the first days of disease [19, 33].…”

“…The complex factor VIII (FVIII) and vWF improve platelets aggregation and clot stability [33]. Increased levels of vWF were demonstrated in DVI patients during the first days of disease [19, 33]. FVIII/vWF complex is likely to play a key hemostatic role during the early course of DVI [10, 22].…”

“…Endothelial activation may be responsible for extravascular plasma leakage and shock in severe DVI patients [2–5]. However, in the majority of DVI patients, inflammation and endothelial cell activation may represent a compensatory mechanism for thrombocytopenia, clot impairment and hypocoagulability during the early course of disease due to fibrinogen activation and increased vWF levels, which improve platelet aggregation [33–35]. …”