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von Willebrand factor levels in the diagnosis of von Willebrand disease: a systematic review and meta-analysis
| Content Provider | Scilit |
|---|---|
| Author | O’Donnell, James S. Kalot, Mohamad A. Husainat, Nedaa Alayli, Abdallah El Abughanimeh, Omar Diab, Osama Tayiem, Sammy Madoukh, Bader Dimassi, Ahmad Bilal Qureini, Aref Ameer, Barbara Eikenboom, Jeroen C. J. Giraud, Nicolas McLintock, Claire McRae, Simon Montgomery, Robert R. Scappe, Nikole Sidonio, Robert F. Brignardello-Petersen, Romina Flood, Veronica H. Connell, Nathan T. James, Paula D. Mustafa, Reem A. |
| Copyright Year | 2021 |
| Description | Von Willebrand disease (VWD) is associated with significant morbidity as a result of excessive mucocutaneous bleeding. Early diagnosis and treatment are important to prevent and treat these symptoms. We systematically reviewed the accuracy of diagnostic tests using different cutoff values of von Willebrand factor antigen (VWF:Ag) and platelet-dependent von Willebrand factor (VWF) activity assays in the diagnosis of VWD. We searched Cochrane Central Register for Controlled Trials, MEDLINE, and Embase databases for eligible studies. We pooled estimates of sensitivity and specificity and reported patient-important outcomes when relevant. This review included 21 studies that evaluated VWD diagnosis. The results showed low certainty in the evidence for a net health benefit from reconsidering the diagnosis of VWD vs removing the disease diagnosis in patients with VWF levels that have normalized with age. For the diagnosis of type 1 VWD, VWF sequence variants were detected in 75% to 82% of patients with VWF:Ag < 0.30 IU/mL and in 44% to 60% of patients with VWF:Ag between 0.30 and 0.50 IU/mL. A sensitivity of 0.90 (95% confidence interval [CI], 0.83-0.94) and a specificity of 0.91 (95% CI, 0.76-0.97) were observed for a platelet-dependent VWF activity/VWF:Ag ratio < 0.7 in detecting type 2 VWD (moderate certainty in the test accuracy results). VWF:Ag and platelet-dependent activity are continuous variables that are associated with an increase in bleeding risk with decreasing levels. This systematic review shows that using a VWF activity/VWF:Ag ratio < 0.7 vs lower cutoff levels in patients with an abnormal initial VWD screen is more accurate for the diagnosis of type 2 VWD. |
| Related Links | https://ashpublications.org/bloodadvances/article-pdf/doi/10.1182/bloodadvances.2021005430/1826316/bloodadvances.2021005430.pdf |
| Ending Page | 71 |
| Page Count | 10 |
| Starting Page | 62 |
| DOI | 10.1182/bloodadvances.2021005430 |
| Journal | Blood advances |
| Issue Number | 1 |
| Volume Number | 6 |
| Language | English |
| Publisher | American Society of Hematology |
| Publisher Date | 2021-12-30 |
| Access Restriction | Open |
| Subject Keyword | Hematology Bleeding Antigen Vwf Vwd Systematic Cutoff Certainty Diagnosis of Type Treatment Journal: Blood advances (Vol- 1, Issue- 1) |
| Content Type | Text |
| Resource Type | Article |