Gastrointestinal bleeding from angiodysplasia in von Willebrand disease: Improved diagnosis and outcome prediction using videocapsule on top of conventional endoscopy

2020 ◽  
Author(s):  
Antoine Rauch ◽  
Camille Paris ◽  
Yohann Repesse ◽  
Julien Branche ◽  
Roseline D’Oiron ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Outcome Prediction ◽  
Von Willebrand Disease ◽  
Conventional Endoscopy ◽  
Von Willebrand

Von Willebrand disease and gastrointestinal bleeding: A national inpatient sample study

2019 ◽  
Vol 178 ◽  
pp. 119-123 ◽  
Author(s):  
Anastasia Tsagianni ◽  
Diane M. Comer ◽  
Jonathan G. Yabes ◽  
Margaret V. Ragni
Keyword(s):  
Gastrointestinal Bleeding ◽  
Von Willebrand Disease ◽  
National Inpatient Sample ◽  
Von Willebrand

A Two-Year Prospective Study on Bleeding Tendency In 105 Patients with Type 2 A and M Von Willebrand Disease

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 543-543
Author(s):  
Giancarlo Castaman ◽  
Augusto B. Federici ◽  
Luciano Baronciani ◽  
Pier M. Mannucci ◽  
Francesco Rodeghiero
Keyword(s):  
Molecular Weight ◽  
Gastrointestinal Bleeding ◽  
High Molecular Weight ◽  
Von Willebrand Disease ◽  
Bleeding Tendency ◽  
Bleeding Episodes ◽  
Von Willebrand ◽  
High Molecular Weight Multimers

Abstract Abstract 543 Background: Type 2 A and 2 M von Willebrand Disease (VWD) are both characterized by the presence of a dysfunctional von Willebrand factor (VWF) and a variable bleeding tendency. The bleeding incidence and possible clinical differences between these two types have never been investigated prospectively in a large number of patients. Aims and design of the study: To investigate clinical history and determinants of bleeding in a cohort of patients (pts) with type 2 A and M VWD characterized by mutations over 24 months of follow-up. Patients and Methods: 10 families with type 2 A VWD (47 patients) and 15 with type 2 M (58 patients) with type 2 M VWD were diagnosed according to the recommendations of the ISTH-SSC subcommittee on VWF and prospectively followed-up from April 2007 to March 2009. Bleeding score (BS) was calculated in all the patients at enrollment by the administration of the same questionnaire tested in type 1 VWD. In case of VWF:RCo levels < 10 U/dL, all patients were tested with a more sensitive ELISA assay to calculate ratios between VWF:RCo and VWF:Ag. VWF mutations were identified in all the patients. Results: The 105 patients were characterized by the following mutations: type 2 A R202W/R1583Q 1 pt, S1506L 11 pts, S1543F 1 pt, R1596W 3 pts, V1607D 8 pts, I1628T 1 pt, G1629R 1 pt, G1631D 9 pts, V1665E 8 pts, Q2520P 2 pts, multiple changes 1 pt. Type 2 M: L1278P 6 pts, R1315L 3 pts, R1315C 9 pts, Y1321C 9 pts, L1361W 4 pts, R1374H 23 pts, C1927/c.8155+6C>T 1 pt, c.3831del-3 3 pts. The table summarizes the main demographic and laboratory characteristics. All the patients had a VWF:RCo/VWF:Ag ratio <0.6. The mean BS and VWF:Ag were significantly higher in type 2 A (P < 0.01). No correlation between VWF:RCo levels and the severity of BS was observed. During follow-up, the bleeding episodes in patients with type 2 M and R1374H or R1315C mutations were very few and mild while they were recurrent and severe in those with type 2 A and V1665E or G1631D mutations, the only laboratory difference being the lack of high molecular weight multimers in type 2 A VWD. Furthermore, 44 gastrointestinal bleeding episodes occurred in 15 patients with type 2 A (range 1–7) compared to 8 episodes in 2 patients with type 2 M (range 2–6). Older age was strongly related to the risk of recurrence of this type of bleeding. Conclusions: Bleeding tendency in type 2 A VWD is higher than that of type 2 M VWD and it is not related to the reduction of FVIII:C and VWF activity. The risk of gastrointestinal bleeding is greater in type 2 A and might be related to the lack of high molecular weight multimers and older age. Further analysis are required to evaluate the definite impact of some mutations on the different bleeding tendency. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Angiodysplasia in von Willebrand Disease: Understanding the Clinical and Basic Science

2017 ◽  
Vol 43 (06) ◽  
pp. 572-580 ◽  
Author(s):  
Soundarya Selvam ◽  
Paula James
Keyword(s):  
Gastrointestinal Bleeding ◽  
Molecular Pathology ◽  
Von Willebrand Disease ◽  
Clinical Implications ◽  
The Past ◽  
Vessel Development ◽  
Novel Target ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Disease Understanding

AbstractSevere and intractable gastrointestinal bleeding caused by angiodysplasia is a debilitating problem for up to 20% of patients with von Willebrand disease (VWD). Currently, the lack of an optimal treatment for this recurrent problem presents an ongoing challenge for many physicians in their management of affected patients. Over the past few years, studies have pointed to a regulatory role for the hemostatic protein, von Willebrand factor (VWF), in angiogenesis, providing a novel target for the modulation of vessel development. This article will review the clinical implications and molecular pathology of angiodysplasia in VWD.

Gastrointestinal bleeding due to angiodysplasia in patients with type 1 von Willebrand disease: report on association and management

Haemophilia ◽  
2007 ◽  
Vol 14 (1) ◽  
pp. 150-152 ◽  
Author(s):  
S. SIRAGUSA ◽  
A. MALATO ◽  
L. LO COCO ◽  
V. CIGNA ◽  
G. SACCULLO ◽  
...  
Keyword(s):  
Gastrointestinal Bleeding ◽  
Von Willebrand Disease ◽  
Von Willebrand

Gastrointestinal angiodysplasia and bleeding in von Willebrand disease

2014 ◽  
Vol 112 (09) ◽  
pp. 427-431 ◽  
Author(s):  
Massimo Franchini ◽  
Pier Mannuccio Mannucci
Keyword(s):  
Gastrointestinal Bleeding ◽  
Von Willebrand Factor ◽  
Severe Symptom ◽  
Bleeding Disorder ◽  
Von Willebrand Disease ◽  
Diagnosis And Treatment ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Mucocutaneous Bleeding

SummaryVon Willebrand disease (VWD), the most common genetic bleeding disorder, is characterised by a quantitative or qualitative defect of von Willebrand factor (VWF). Patients with VWD suffer from mucocutaneous bleeding, of severity usually proportional to the degree of VWF defect. In particular, gastrointestinal bleeding associated with angiodysplasia is often a severe symptom of difficult management. This review focuses on the pathophysiology, diagnosis and treatment of VWD-associated gastrointestinal angiodysplasia and related bleeding.

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