Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy

Abstract

Key Points In children undergoing tonsillectomy, low VWF levels did not predict surgical bleeding in the absence of major bleeding history. Children have lower VWF levels than adults and these lower levels do not appear to be associated with bleeding.

Association of Blood Type With Postsurgical Mucosal Bleeding in Pediatric Patients Undergoing Tonsillectomy With or Without Adenoidectomy

“…However, the number of patients in the low VWF and disease categories, especially among those who experienced bleeding, was very small and may have influenced our ability to capture any differences. Gill et al 27 also noted the transient nature of low VWF values, especially in children. Interestingly, we found that nearly one-third of patients who had a known BT and were categorized as low VWF in our study had a subsequent normal VWD panel result prior to their procedure, did not receive treatment based on clinician or family preference, and did not have bleeding postoperatively.…”

Low VWF: insights into pathogenesis, diagnosis, and clinical management

“…15 Studies have demonstrated that many patients with reduced VWF levels in the 30 to 50 IU/dL range do not have a significant bleeding phenotype. 61 Consequently, a critical first step in diagnosis is to objectively assess bleeding using a validated BAT. The 2 scores most widely used in this context are the ISTH and condensed MCMDM-1 VWD BATs.…”

“…Thus, although reduced VWF levels in the 30 to 50 IU/dL range may constitute a risk factor for bleeding, they may not be sufficient to cause a bleeding phenotype in the absence of other hemostatic factors. 14,61,62 However, it also seems likely that a significant cohort of patients with low VWF and bleeding complications (particularly women with HMB) is not being diagnosed. 18 Given the significant associated morbidity, it is imperative that awareness among the general population and health care professionals is actively promoted.…”

“…For example, Flood et al recently reported that bleeding complications were rare in pediatric patients with low VWF undergoing tonsillectomy. 61 Treatment options for patients with low VWF include antifibrinolytic agents (tranexamic acid or aminocaproic acid), DDAVP, and VWF-containing concentrates. 63,64 Although reduced VWF biosynthesis and subtle enhanced clearance have been implicated in the pathogenesis of low VWF, recent studies have reported that the vast majority of patients with low VWF demonstrate excellent and sustained increases in plasma VWF levels following DDAVP administration.…”

Low von Willebrand factor in pediatric patients: Retrospective analysis of 293 cases informs diagnostic and therapeutic decision making

“…Low von Willebrand factor (VWF) is an intermediate reduction in plasma VWF levels (30‐49%) and is generally considered a risk factor for bleeding but not a bleeding disorder 1‐3 . Low VWF poses a number of diagnostic and therapeutic challenges for both patients and providers since individuals report heterogeneous bleeding phenotypes.…”