ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease
| Autor: | Robert R. Montgomery, Jeroen Eikenboom, Barbara Ameer, Sandra L. Haberichter, Mohamad A. Kalot, Nedaa Husainat, Barbara A. Konkle, Paula D. James, Reem A. Mustafa, James S. O’Donnell, Vicki Jacobs-Pratt, Nikole Scappe, Claire McLintock, Robert F. Sidonio, Veronica H. Flood, Simon McRae, Nicolas Giraud, Nathan T. Connell, Jorge Di Paola |
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| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
medicine.diagnostic_test business.industry MEDLINE Hematology Guideline 030204 cardiovascular system & hematology medicine.disease Bleeding diathesis 03 medical and health sciences 0302 clinical medicine Family medicine Health care medicine Von Willebrand disease Implementation research business Grading (education) Clinical Guidelines 030215 immunology Genetic testing |
| Zdroj: | Blood Adv Blood Advances, 5(1), 280-300. AMER SOC HEMATOLOGY |
| Popis: | Background: von Willebrand disease (VWD) is the most common inherited bleeding disorder known in humans. Accurate and timely diagnosis presents numerous challenges. Objective: These evidence-based guidelines of the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) are intended to support patients, clinicians, and other health care professionals in their decisions about VWD diagnosis. Methods: ASH, ISTH, NHF, and WFH established a multidisciplinary guideline panel that included 4 patient representatives and was balanced to minimize potential bias from conflicts of interest. The Outcomes and Implementation Research Unit at the University of Kansas Medical Center (KUMC) supported the guideline-development process, including performing or updating systematic evidence reviews up to 8 January 2020. The panel prioritized clinical questions and outcomes according to their importance for clinicians and patients. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE Evidence-to-Decision frameworks, to assess evidence and make recommendations, which were subsequently subject to public comment. Results: The panel agreed on 11 recommendations. Conclusions: Key recommendations of these guidelines include the role of bleeding-assessment tools in the assessment of patients suspected of VWD, diagnostic assays and laboratory cutoffs for type 1 and type 2 VWD, how to approach a type 1 VWD patient with normalized levels over time, and the role of genetic testing vs phenotypic assays for types 2B and 2N. Future critical research priorities are also identified. |
| Databáze: | OpenAIRE |
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