Laboratory assays of VWF activity and use of desmopressin trials in the diagnosis of VWD: a systematic review and meta-analysis

Author:

Kalot Mohamad A.1ORCID,Husainat Nedaa2,Abughanimeh Omar3ORCID,Diab Osama4,El Alayli Abdallah5,Tayiem Sammy6,Madoukh Bader7,Dimassi Ahmad8ORCID,Qureini Aref9,Ameer Barbara10ORCID,Eikenboom Jeroen11,Giraud Nicolas12,Haberichter Sandra13,Jacobs-Pratt Vicky14,Konkle Barbara A.15ORCID,McRae Simon16,Montgomery Robert1417,O’Donnell James S.18ORCID,Brignardello-Petersen Romina19ORCID,Flood Veronica17,Connell Nathan T.20ORCID,James Paula21ORCID,Mustafa Reem A.5ORCID

Affiliation:

1. 1Department of Internal Medicine, State University of New York at Buffalo, Buffalo, NY;

2. 2Department of Internal Medicine, St. Mary's Hospital, St. Louis, MO;

3. 3Division of Oncology and Hematology, University of Nebraska Medical Center-Fred & Pamela Buffett Cancer Center, Omaha, NE;

4. 4Department of Hematology,

5. 5Department of Nephrology and Hypertension, Outcomes and Implementation Research Unit, and

6. 6Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS;

7. 7Department of Internal Medicine, State University of New York-Upstate Medical University, Syracuse, NY;

8. 8Department of Internal Medicine, Lebanese American University, Achrafiye, Beirut, Lebanon;

9. 9Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS;

10. 10Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ;

11. 11Division of Thrombosis and Hemostasis, Department of Internal Medicine, Leiden University Medical Center, Leiden, The Netherlands;

12. 12Association française des hémophiles (AFH), Paris, France;

13. 13Versiti-Blood Center of Wisconsin, Milwaukee, WI;

14. 14Infinity Way, Auburn, ME;

15. 15Division of Hematology, University of Washington, Seattle, WA

16. 16Royal Adelaide Hospital, Adelaide, Australia;

17. 17Division of Hematology/Oncology, Department of Pediatrics, Medical College of Wisconsin, Wauwatosa, WI;

18. 18Royal College of Surgeons in Ireland, Irish Centre for Vascular Biology, Dublin 2, Ireland;

19. 19Department of Health Research Methods, Evidence, and Impact, McMaster University, Hamilton, ON, Canada;

20. 20Brigham and Women’s Hospital, Harvard Medical School, Boston, MA; and

21. 21Department of Medicine, Queen’s University, Kingston ON, Canada

Abstract

Abstract von Willebrand Disease (VWD) is associated with significant morbidity because of excessive bleeding. Early diagnosis and treatment are important to prevent and treat these symptoms. We systematically reviewed the accuracy of any von Willebrand factor (VWF) activity assay in the diagnosis and classification of patients for VWD. We searched Cochrane Central, MEDLINE, and EMBASE for eligible studies. The risk of bias was assessed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS)-2 and the certainty of evidence using the GRADE framework. We pooled estimates of sensitivity and specificity. The review included 77 studies that evaluated the use of newer tests of VWF platelet binding activity (VWF:GPIbR, VWF:GPIbM) and VWF:RCo for the diagnosis of VWD (13 studies), VWF propeptide to VWF:Ag ratio, and desmopressin trial for the diagnosis of type 1C VWD (5 studies), VWF multimer analysis and VWF:CB/VWF:Ag ratio for the classification of type 2 VWD (11 studies), genetic testing and ristocetin-induced platelet aggregation to diagnose type 2B VWD (14 studies), genetic testing and FVIII:VWF binding to diagnose type 2N VWD (17 studies). Based on available diagnostic test accuracy, there appear to be comparable test accuracy results between newer tests of platelet binding activity of VWF function and VWF:RCo. The findings of these reviews support VWF multimer analysis or VWF:CB/VWF:Ag to diagnose type 2 VWD. The desmopressin trial test with 1- and 4-hour postinfusion blood work is the test of choice to confirm increased VWF clearance in patients with suspected VWD type 1C. Additionally, genetic testing is most useful in diagnosing type 2B VWD and has a role in the diagnostic algorithm of suspected type 2N VWD.

Publisher

American Society of Hematology

Subject

Hematology

同舟云学术

1.学者识别学者识别

2.学术分析学术分析

3.人才评估人才评估

"同舟云学术"是以全球学者为主线,采集、加工和组织学术论文而形成的新型学术文献查询和分析系统,可以对全球学者进行文献检索和人才价值评估。用户可以通过关注某些学科领域的顶尖人物而持续追踪该领域的学科进展和研究前沿。经过近期的数据扩容,当前同舟云学术共收录了国内外主流学术期刊6万余种,收集的期刊论文及会议论文总量共计约1.5亿篇,并以每天添加12000余篇中外论文的速度递增。我们也可以为用户提供个性化、定制化的学者数据。欢迎来电咨询!咨询电话:010-8811{复制后删除}0370

www.globalauthorid.com

TOP

Copyright © 2019-2024 北京同舟云网络信息技术有限公司
京公网安备11010802033243号  京ICP备18003416号-3