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.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/real-life-management-of-digestive-endoscopies-is-there-a-bleeding-risk-for-patients-with-von-willebrand-disease/