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Real-life Management of Digestive Endoscopies: Is There a Bleeding Risk for Patients with von Willebrand Disease?

V. Horvais1, B. Guillet2, P. Beurrier3, V. Cussac4, B. Pan-Petesch5, N. Baty3, S. Cochennec2, G. Drugmanne5, S. Bayart2, J. Rose4, F. Nedelec-Gac2, N. Drillaud1, M. Sigaud1, M. Fouassier1, C. Ternisien1, M. Trossaert1, the BERHLINGO Group

1Nantes University Hospital, Nantes, France, 2Rennes University Hospital, Rennes, France, 3Angers University Hospital, Angers, France, 4Le Mans Hospital, Le Mans, France, 5Brest University Hospital, Brest, France

Abstract Number: PB1550

Meeting: ISTH 2020 Congress

Theme: Platelet Disorders and von Willebrand Disease » VWF and von Willebrand Factor Disorders - Clinical Conditions

Background: Gastrointestinal (GI) bleedings are well-recognised complications of von Willebrand disease (VWD), requiring many endoscopic procedures.

Aims: To report our real-life experience of digestive endoscopies’ management in patients with VWD and identify a potential post-examination hemorrhagic risk.

Methods: A retrospective, non interventional, multicentric study was carried out over 48 months, from the BERHLINGO database, in 5 hemophilia centers in Western France. Types of endoscopies, VWD and treatments were assessed.

Results: The analysis phase is currently going on and data presented here are for the coordinating centre cohort only.
So far, among a cohort of 481 patients, 207 were hospitalized during 439 in-stays. Notably, 92 GI in-stays were identified: 48 involve digestive endoscopies, for 37 patients (Cf table 1).

  Patients: n (%) In-stays: n (%) Age: mean [Min;Max] Mean basal VWF Activity (IU.L-1) Mean basal FVIII:C (IU.L-1)
Type non classified 1 (2.7%) 1 (2.1%) 46.9 [46.9;46.9] 19.0 27.0
Type 1 5 (13.5%) 5 (10.4%) 47.9 [29.0;63.7] 25.0 52.0
Type 2 (Total) 26 (70.3%) 33 (68.7%) 56.0 [18.9;78.4] 22.5 38.3
Sub-type 2 unclassified 2 (5.4%) 3 (6.2%) 61.7 [58.6;63.1] 8.3 28.7
Sub-type 2A 5 (13.5%) 5 (10.4%) 60.3 [56.2;66.9] 20.4 50.0
Sub-type 2B 5 (13.5%) 9 (18.8%) 56.7 [44.6;63.0] 20.7 45.2
Sub-type 2M 11 (29.8%) 13 (27.1%) 50.7 [18.9;78.4] 11.8 34.7
Sub-type 2N 3 (8.1%) 3 (6.2%) 64.6 [57.9;71.4] 89.0 21.7
Type 3 5 (13.5%) 9 (18.8%) 50.7 [21.7;68.0] < 1.9 1.5

[Table 1: patients’ characteristics]

An ileocolonoscopy alone was performed in 54.2% of cases (n = 26), a upper endoscopy alone in 20.8% of cases (n = 10) and a combination of several procedures in 25.0% of cases (n = 12).
DDAVp (desmopressin) was administered in 27.1% of cases (n = 13), mainly for types 1 and 2M vWD. Without biopsies (n = 6), the mean DDAVp dosage was 0.25 µg/kg/injection during 1.0 day, whereas it was 0.26 µg/kg/injection with 1.19 injection/day during 2.4 days in case of biopsies (n=7).
von Willebrand Factor (VWF) was administered in 56.2% of cases (n = 27), essentially for type 2 (63.0%) and type 3 (33.3%) VWD (Cf table 2). Without biopsies (n = 17), the mean VWF dosage was 44.1 IU/kg/ED (exposure day) during 2.3 ED whereas it was 45.9 IU/kg/ED during 4.1 ED in case of biopsies (n = 10).

  Patients: n Treatments (in-stays, n) Mean basal VWF Activity (IU.L-1) [Min; Max] Mean basal FVIII:C (IU.L-1) [Min; Max] Pre-endoscopy VWF (IU/kg/inj) [Min; Max] Pre-endoscopy FVIII (IU/kg/inj) [Min; Max] Mean VWF dosage (IU/kg/ED) [Min; Max] Mean FVIII dosage (IU/kg/ED) [Min; Max]
Type 1 1 VWF+FVIII, n=1 14 [14; 14] 41 [41; 41] 47.4 [47.4; 47.4] 19.7 [19.7; 19.7] 36.8 [36.8; 36.8] 15.4 [15.4; 15.4]
Type 2 (Total) 13 / 15.0 [4.0; 38.0] 41.0 [17.0; 80.0] 30.9 [0.0; 62.1] 11.7 [0.0; 25.9] 37.9 [21.2; 62.1] 14.0 [0.0 ; 25.9]
Sub-type 2A 1 VWF+FVIII, n=1 24.0 [24.0; 24.0] 50.0 [50.0; 50.0] 0.0 [0.0; 0.0] 0.0 [0.0; 0.0] 37.9 [37.9; 37.9] 15.8 [15.8; 15.8]
Sub-type 2B 3 VWF only, n=2 VWF+FVIII, n=4 20.0 [11.0; 38.0] 46.2 [34.0; 80.0] 34.4 [0.0; 44.1] 11.3 [0.0; 18.2] 40.2 [23.4; 54.5] 12.0 [0.0; 22.7]
Sub-type 2M 9 VWF+FVIII, n=10 10.8 [4.0; 19.0] 36.5 [17.0; 50.0] 32.0 [0.0; 62.1] 13.3 [0.0; 25.9] 36.4 [21.2; 62.1] 15.2 [8.8; 25.9]
Type 3 5 VWF+FVIII, n=9 <1.9 [<1.0; <3.0] 1.5 [1.0; 2.0] 43.1 [0.0; 60.0] 15.2 [0.0; 30.0] 52.4 [33.3; 84.0] 24.3 [5.0; 80.0]

[Table 2: patients’ VWF treatments]

Concomitantly, all patient were prescribed per os tranexamic acid. No patient was readmitted within 15 days of the endoscopy.

Conclusions: The implementation of registries with real-life data could assess clinical practices and safety in patients with VWD undergoing endoscopy, and ensure compliance with guidelines.

To cite this abstract in AMA style:

Horvais V, Guillet B, Beurrier P, Cussac V, Pan-Petesch B, Baty N, Cochennec S, Drugmanne G, Bayart S, Rose J, Nedelec-Gac F, Drillaud N, Sigaud M, Fouassier M, Ternisien C, Trossaert M, the BERHLINGO Group . Real-life Management of Digestive Endoscopies: Is There a Bleeding Risk for Patients with von Willebrand Disease? [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/real-life-management-of-digestive-endoscopies-is-there-a-bleeding-risk-for-patients-with-von-willebrand-disease/. Accessed October 1, 2023.

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