New international clinical practice guidelines have been published for the diagnosis and management of von Willebrand Disease (VWD) , the most common inherited bleeding disorder.
The guidelines were drawn up by The American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the US National Hemophilia Foundation (NHF), and World Federation of Hemophilia (WFH) and are published in Blood Advances.
Two expert panels made up of international haematologists, people living with VWD and scientists with expertise in appraising clinical evidence developed the guidelines. They started by identifying the most important clinical questions about VWD diagnosis and treatment through an international survey and panel discussions.
This was combined with a systematic review of all available evidence about VWD, conducted by the University of Kansas Medical Center, USA, which the panel referred to when making its recommendations.
The guidelines detail 19 recommendations on diagnosis and management of the disease.
For the first time, it is recommended that VWD patients who suffer from frequent, severe bleeding that decreases their quality of life should receive routine VWD prophylaxis, an injectable concentrate of clotting protein, several times a week.
The guidelines also call for the classification of VWD to be more inclusive of patients who experience bleeding, but whose blood tests do not meet currently accepted thresholds for diagnosis.
Dr Martin Tallman, from Memorial Sloan Kettering Cancer Center, USA and 2021 ASH President, said: “While VWD is a common bleeding disorder, it is also complex, presenting challenges in the timely diagnosis and appropriate management of bleeding for patients.
“Because diagnosis is not straightforward and symptoms range in severity, there is a need for trustworthy guidelines to help improve the quality of care for patients.”
Dr Leonard Valentino, president of NHF, added: “These guidelines are an extremely important step in our quest to address the difficulties individuals go through to obtain an accurate, timely diagnosis, and appropriate treatment.
“The challenge ahead will be for us to educate both those living with VWD and healthcare professionals on the guideline recommendations.”
The guidelines are accompanied by tools and educational resources to help patients, haematologists and other health care providers understand and implement the recommendations.
James PD, Connell NT, Ameer B, Di Paola J, Eikenboom J, Giraud N, Haberichter S, Jacobs-Pratt V, Konkle B, McLintock C, McRae S, Montgomery RR, O’Donnell JS, Scappe N, Sidonio, Jr, R, Flood VH, Husainat N, Kalot MA, Mustafa, RA. (2021) “ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease.” Blood Adv, doi: 10.1182/bloodadvances.2020003265
Connell NT, Flood VH, Brignardello-Petersen R, Abdul-Kadir R, Arapshian A, Couper S, Grow JM, Kouides P, Laffan M, Lavin M, Leebeek FWG, O’Brien SH, Ozelo MC, Tosetto A, Weyand AC, James PD, Kalot MA, Husainat N, Mustafa RA. (2021) “ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease.” Blood Adv, doi: 10.1182/bloodadvances.2020003264
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