Abstract Number: PB0931
Meeting: ISTH 2021 Congress
Theme: Platelet Disorders, von Willebrand Disease and Thrombotic Microangiopathies » VWF and von Willebrand Factor Disorders - Clinical Conditions
Background: Qualitative or quantitative defects of Von Willebrand Factor (VWF) such as in Von Willebrand Disease (VWD) are associated with vascular abnormalities, especially in the gastrointestinal (GI) tract. However, the locations, extent, and natural history of these abnormalities in patients with VWD is not well understood.
Aims: In order to summarise the existing literature on the topic we conducted a scoping review of vascular abnormalities in patients with VWD.
Methods: We searched MEDLINE and EMBASE from inception to September 1st, 2020 for studies clinically describing vascular malformations in VWD patients. Screening and data extraction was completed independently and in duplicate. If more than one site of malformation was reported in a patient, each was documented individually.
Results: After screening, 54 studies reported patient-level data comprising 146 patients were included. Patient information is detailed in Table 1. Type 2A (39%) and Type 3 (14.4%) were the most common VWD subtypes. The most common site of vascular malformation was the GI tract, occurring in 124 patients (84.9%), while 18 (12.3%) had non-GI vascular abnormalities and 4 (2.7%) had both GI and non-GI vascular abnormalities. Fifteen patients also had a diagnosis of Hereditary Hemorrhagic Telangiectasia. Angiodysplasia was most commonly diagnosed with endoscopy (71 conventional, 37 video capsule, 11 push endoscopy). Treatment for bleeding angiodysplasia was varied and included statins (19 patients), tamoxifen (5 patients), thalidomide (10 patients), surgery (18 patients), and endoscopic interventions (42 patients). With respect to outcomes 3 patients (2%) died, 53 (36.2%) had bleeding resolve, and 9 (6.2%) had ongoing bleeding. Outcomes were not specified in the majority (55.5%) of patients.
| N=146 patients | |
| Gender Male Female Unspecified |
71 (48.6%) 56 (38.4%) 19 (13%) |
| VWD Type Type 1 Type 2A Type 2B Type 2M Type 2N Type 2 Unspecified Type 3 Unspecified |
17 (11.6%) 57 (39%) 18 (12.3%) 11 (7.5%) 1 (0.7%) 2 (1.4%) 21 (14.4%) 19 (13%) |
Demographics and disease characteristics of included patients
Conclusions: Vascular abnormalities in patients with VWD occur predominantly in the gastrointestinal tract and in patients with Type 2 or Type 3 VWD. The clinical treatment and natural history of these abnormalities remain understudied and further research is needed.
To cite this abstract in AMA style:
Chornenki N, Shanjer M, James P. Vascular Abnormalities in Patients with Von Willebrand Disease: A Scoping Review [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 2). https://abstracts.isth.org/abstract/vascular-abnormalities-in-patients-with-von-willebrand-disease-a-scoping-review/. Accessed July 1, 2022.« Back to ISTH 2021 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/vascular-abnormalities-in-patients-with-von-willebrand-disease-a-scoping-review/