Abstract Number: PB1560
Meeting: ISTH 2020 Congress
Theme: Platelet Disorders and von Willebrand Disease » VWF and von Willebrand Factor Disorders - Clinical Conditions
Background: Hemophilia Treatment Centers (HTC) are specialized centers that provide comprehensive care for patients with chronic bleeding disorders. While it has been shown that hemophilia patients receiving care at HTCs experience decreased morbidity and mortality, little research has been done on the outcomes of patients with von Willebrand disease (VWD).
Aims: To compare the quality of peri-procedural care received by patients with VWD at HTCs vs non-HTCs.
Methods: Patients with a diagnosis of VWD meeting inclusion and exclusion criteria were abstracted from the electronic medical record. Comparisons were performed according to the setting of care, HTC vs non-HTC, at the time of the invasive procedure. Outcomes included frequency of post-procedure bleeding, procedural estimated blood loss (EBL), and peri-procedural VWD-specific therapy use per National Heart, Lung, and Blood Institute (NHLBI) guidelines. Mixed-effects models incorporated within-person dependencies.
Results: There were 681 invasive procedures performed on 310 patients with 9% performed at HTCs. Cases at HTCs were younger with more severe VWD (Table 1). VWD-specific therapy was used per NHLBI guidelines in 100% of HTC cases compared with 10.6% of non-HTC cases. DDAVP, 96.0%, and Humate-P, 78.7%, were the primary therapies of choice in non-HTC and HTC cases, respectively. In 66.6% of non-HTC cases, “no treatment” was the principal reason for not following NHLBI guidelines. There was no difference in frequency of post-procedure bleeding or procedural EBL (Table 2).
Conclusions: Despite the widespread peri-procedural use of VWD-specific therapy outside established guidelines at non-HTCs, there was no difference in frequency of post-procedure bleeding or procedural EBL between HTCs and non-HTCs. Possible explanations include fewer patients with severe VWD and higher, even normal, VWF levels at non-HTCs. In conclusion, compared to VWD patients at non-HTCs, those receiving care at HTCs are more severe and more likely to receive peri-procedural VWD-specific therapy per recommended guidelines, and with no greater bleed risk.
| 1Per procedure | HTC (N=61) | Non-HTC (N=620) | p-value |
| Age, mean (SD) | 43.8 (14.6) | 52.6 (17.0) | 0.033 |
| Sex – Male, Female (%) | 16 (14.8), 45 (73.8) | 123 (19.8), 497 (80.2) | 0.66 |
| 2Race – White, Black, Asian (%) | 53 (89.8), 6 (10.2), 0 (0.0) | 565 (91.7), 45 (7.3), 6 (1.0) | 30.22 |
| 4Ethnicity – Non-Hispanic, Hispanic (%) | 57 (100.0), 0 (0.0) | 607 (99.4), 4 (0.6) | 5– |
| 6VWD type – 1, 2A, 2B, 2M, 2N, 3 (%) | 43 (70.5), 1 (1.6), 0 (0.0), 0 (0.0), 1 (1.6), 16 (26.2) | 421 (98.6), 3 (0.7), 0 (0.0), 3 (0.7), 0 (0.0), 0 (0.0) | 7 <0.001 |
| 8VWF antigen and ristocetin cofactor (IU/dL), mean (SD) | 0.61 (0.44), 0.39 (0.31) | 0.91 (0.50), 0.61 (0.40) | 0.049, 0.032 |
| Major invasive procedure, Minor invasive procedure (%) | 14 (23.0), 47 (77.0) | 142 (22.9), 478 (77.1) | 0.34 |
| Comorbidities – Alcohol abuse, Chronic liver disease, Chronic kidney disease, Malignancy (%) | 13 (21.3), 0 (0.0), 3 (4.9), 5 (8.2) | 14 (2.3), 25 (4.0), 25 (4.0), 46 (7.4) | <.001, 5-, 0.56, 0.96 |
| 1All patient characteristics are per procedure except VWF antigen and ristocetin cofactor. 2Race was not available in 2 HTC and 4 non-HTC cases. 3P-value tested white vs. non-white. 4Ethnicity was not available in 4 HTC and 9 non-HTC cases. 5Statistical comparison not appropriate. 6VWD type was not available in 193 non-HTC cases. 7P-value tested type 1 VWD vs non-type 1 VWD. 8VWF antigen and ristocetin cofactor are based on lowest recorded values available. | |||
[Table 1]
| HTC (N=61) | Non-HTC (N=621) | Unadjusted OR (95% CI) | Adjusted OR (95% CI) | |
| 1Post procedure bleeding (%) | 2 (3.3) | 22 (3.5) | 1.03 (0.22-4.90) | 0.98 (0.17-5.72) |
| 2Estimated blood loss >=200 mL (%) | 7 (11.5) | 30 (4.8) | 2.53 (1.00-6.38) | 2.14 (0.76-6.04) |
| 3VWD-specific therapy use per NHLBI guidelines (%) | 61 (100.0) | 66 (10.6) | 4– | 4– |
| 1Adjusted for age, sex, VWF antigen and ristocetin cofactor, alcohol abuse, chronic liver disease, and chronic kidney disease. 2Adjusted for age, sex, VWF antigen and ristocetin cofactor, alcohol abuse, and malignancy. 3VWD-specific therapy refers to DDAVP, VWF concentrates, and antifibrinolytics. 4Statistical comparison not appropriate. | ||||
[Table 2]
To cite this abstract in AMA style:
Seaman C, Bertolet M, Zhang J, Ragni M. Quality of Peri-Procedural Care in Patients with von Willebrand Disease [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/quality-of-peri-procedural-care-in-patients-with-von-willebrand-disease/. Accessed October 1, 2023.« Back to ISTH 2020 Congress
ISTH Congress Abstracts - https://abstracts.isth.org/abstract/quality-of-peri-procedural-care-in-patients-with-von-willebrand-disease/