Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors’ Organisation and the British Society for Haematology

Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors’ Organisation and the British Society for Haematology

Sean Platton1; Peter Baker2; Annette Bowyer3; Catriona Keenan4; Caroline Lawrence5; Will Lester6;Anne Riddell7; Megan Sutherland8

1-Royal London Hospital Haemophilia Centre, London, UK

2-Oxford Haemophilia and Thrombosis Centre, Nuffield Orthopaedic Hospital, Oxford, UK

3-Department of Coagulation, Royal Hallamshire Hospital, Sheffield, UK

4-Department of Haematology & the National Coagulation Centre, St. James’s Hospital, Dublin, Ireland

5-Department of Haematology, Glasgow Royal Infirmary, Glasgow, UK

6-Haemophilia Unit, University Hospitals, Birmingham, UK

7-Katharine Dormandy Haemophilia Centre, Royal Free Hospital, London, UK

8-North West Genomic Laboratory Hub, Manchester University NHS Foundation Trust, Manchester, UK

SUMMARY

This guideline updates the previous guidelines1,2 published on behalf of the British Society for Haematology (BSH) and the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO), focussing on the laboratory components of diagnosis and monitoring. Clinical aspects will be addressed in a separate guideline.

METHODOLOGY

This guideline was compiled according to the BSH process at https://b-s-h.org.uk/media/16732/bsh-guidance-development-process-dec-5-18.pdf. The writing group, which comprised selected members of the BSH Haemostasis and Thrombosis Task Force (BSH HTTF), the UKHCDO Laboratory Working Party (LWP) and members of the UKHCDO Genetics Laboratory Network (GLN), produced the first draft of the manuscript. A literature search was carried out using the terms given in Table S1. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate the levels of evidence and to assess the strength of recommendations. The GRADE criteria can be found at http://www.gradeworkinggroup.org and is summarised in appendix 3 of the guidance document linked above.

Review of the manuscript

Review of the manuscript was performed by the BSH HTTF, the BSH Guidelines Committee and the sounding board of BSH. It was circulated to members of the UKHCDO LWP and GLN, and was on the members section of the BSH website for comment.