vWDben

vWDben is a fictional variant of von Willebrand disease used in educational materials to illustrate milder, mildly atypical presentations of bleeding disorders. The term combines vWD, the acronym for von Willebrand disease, with ben, indicating a benign-appearing variant created for teaching purposes. In this construct, vWDben presents with mild mucocutaneous bleeding symptoms while laboratory parameters remain near normal.

Pathophysiology in this hypothetical model involves a modest reduction in functional von Willebrand factor due to a notional mutation in the VWF gene. This can lead to impaired platelet adhesion and decreased stability of factor VIII, but in vWDben the effects are generally less pronounced than in classic, defined subtypes of von Willebrand disease. The result is a bleeding tendency that is typically mild and primarily mucosal.

Clinical features commonly described for vWDben include easy bruising, occasional epistaxis, and light menorrhagia in susceptible individuals. Platelet counts are usually normal, and coagulation screening may be unrevealing or show only mild abnormalities. Specialized testing, such as von Willebrand factor antigen and ristocetin cofactor activity, may show mild reductions, while genetic testing would reveal a hypothetical VWF variant used for instructional purposes.

Diagnosis and management in the vWDben framework follow educational guidelines rather than clinical practice. Diagnosis relies on a combination of history, mild laboratory deviations, and the exclusion of other bleeding disorders. Management emphasizes avoidance of NSAIDs, use of desmopressin (DDAVP) to provoke endogenous VWF release in teaching scenarios, and antifibrinolytics for minor bleeds. In procedures or significant bleeding, with the caveat that this is a fictional variant, VWF-containing concentrates may be employed in teaching cases.

See also: von Willebrand disease, hemostasis, medical education case studies.