2026-07-06 | Navigating hemostasis: anesthetic challenges in a pituitary tumor with type 2 von Willebrand disease—case report

Introduction The anesthetic management of patients with von Willebrand disease (VWD) present a significant perioperative challenge due to the high risk of hemorrhage. This risk is further elevated in neurosurgical procedures, such as transsphenoidal surgery (TSS) for pituitary neuroendocrine tumors (PitNETs), due to the close proximity to critical neurovascular structures. Case presentation This report describes a 42-year-old male with type 2 VWD and hypothyroidism, diagnosed with a Knosp grade 3A PitNET, who underwent an endoscopic endonasal transsphenoidal resection. A multidisciplinary approach was implemented, including hemodynamic monitoring and targeted replacement of VWF/FVIII concentrate calculated according to plasma levels, maintaining paremeters of VWF ristocetin cofactor/activity (VWF:RCo) (%) and FVIII (%): >80% during the intraoperative period and >50% during the postoperative period. The postoperative course was favorable, with no neurovascular, hemostatic, hemodynamic, or endocrine complications. Discussion The coexistence of VWD and a macro-PitNET creates a profound clinical dilemma that exposes the limitations of conventional perioperative care. While TSS demands strict hemostatic control due to its proximity to major neurovascular structures like the cavernous sinuses and internal carotid arteries, the type 2 VWD directly threatens clot stability and initial platelet plug formation. Given the scarce literature surrounding its neuroanesthetic management, this case report discusses the successful implementation of an individualized strategy. By integrating targeted plasma-derived VWF/FVIII replacement with real-time thromboelastography (TEG) and advanced hemodynamic monitoring, we demonstrate how a coordinated multidisciplinary approach can successfully mitigate overlapping hemorrhagic and neurosurgical risks. Conclusions This case highlights the importance of individualized planning and comprehensive management based on the American Society of Hematology (ASH), International Society on Thrombosis and Haemostasis (ISTH), National Hemophilia Foundation (NHF), and World Federation of Hemophilia (WFH) (ASH–ISTH–NHF–WFH, 2021) guidelines to optimize outcomes in patients with VWD undergoing high-complexity surgery.

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2026-07-06 | Von Willebrand disease: A century of progress.

One hundred years after the initial description of von Willebrand disease, originally referred to as pseudohemophilia, this article is a tribute to Dr Erik von Willebrand and a testament to the progress in our understanding of von Willebrand factor. Main focuses have been on structure and hemostatic function, as well as the advancements in the diagnosis, genetics, and management of von Willebrand disease. Insightful early observations led to the discovery of the main VWF ligands and interaction domains and the molecular mechanisms controlling these associations in the context of hemostasis. Reflecting these intricate mechanisms, the genetics and diagnosis of von Willebrand disease remain challenging, especially for the mild, quantitative deficiencies. Treatment developments and innovations have historically progressed quite slowly and in the shadow of hemophilia. However, recent patient-centered studies underscoring unmet clinical needs have catalyzed a dynamic and rapidly evolving effort to improve patient care and clinical outcomes.

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2026-07-03 | Are electrical stimulation devices the way forward for addressing heavy menstrual bleeding in women with von Willebrand disease?

1. Heavy menstrual bleeding significantly impacts quality of life for adolescents and adults with von Willebrand disease (VWD). Not everyone with VWD experiences heavy periods – and not every perio...

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