Summary
Definition
History and exam
Key diagnostic factors
- bleeding from minor wounds
- postoperative bleeding
- family history of bleeding
- easy and excessive bruising
- menorrhagia
Other diagnostic factors
- gastrointestinal bleeding
- epistaxis
- blood transfusions
- hemarthrosis
- central nervous system bleeding
- hematuria
Risk factors
- positive family history
- consanguineous relationships
- lymphoproliferative disorders
- aortic stenosis
- myeloproliferative disorders
- hypothyroidism
Diagnostic investigations
1st investigations to order
- prothrombin time (PT)
- activated partial thromboplastin time (APTT)
- CBC
- von Willebrand factor antigen
- von Willebrand factor function assay (ristocetin cofactor and collagen binding assays)
- factor VIII activity
Tests to avoid
- bleeding time test
Investigations to consider
- von Willebrand factor multimer analysis
- platelet aggregometry
- factor VIII - von Willebrand factor binding assay
- TFTs
- serum protein electrophoresis
Treatment algorithm
VWD type unknown with active severe hemorrhage
all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and VWF-containing concentrates
all types VWD with severe bleeding or before high-risk bleeding procedures (including where sustained high levels of VWF required for several days): with or without mucosal involvement
all types VWD with minor bleeding or before procedures: involving mucous membranes
type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate nonmucosal bleeding or before procedures not involving mucous membranes: nonpregnant
type 1, 2A, or 2M VWD: desmopressin nonresponder with moderate mucosal or minor/moderate nonmucosal bleeding or before procedures not involving mucous membranes: nonpregnant
type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: nonpregnant
all types VWD: pregnant
all types VWD with chronic or recurrent menorrhagia
all types VWD with significant chronic or recurrent bleeding or with treatment-refractory menorrhagia
Contributors
Authors
Mike Laffan, DM, FRCP, FRCPath
Professor of Haemostasis and Thrombosis
Imperial College Academic Health Sciences Centre
Hammersmith Hospital
London
UK
Declarações
ML has received consultancy fees from Pfizer, Shire, Portola and Roche, speaker fees from Pfizer and Bayer, and travel support from Bayer and Shire. ML is an author of a number of references cited in this topic.
Agradecimentos
Prof Mike Laffan would like to gratefully acknowledge Dr Barbara A. Konkle, a previous contributor to this topic.
Declarações
BAK declares that she has no competing interests.
Revisores
Margaret Ragni, MD
Director
Hemophilia Center of Western Pennsylvania
Pittsburgh
PA
Divulgaciones
MR is an author of a reference cited in this topic.
David Keeling, BSc, MD, FRCP, FRCPath
Consultant Haematologist and Director
Oxford Haemophilia & Thrombosis Centre
Churchill Hospital
Oxford
UK
Раскрытие информации
DK declares that he has received payments from CSL Behring for giving a lecture and attending an advisory board. He is an author of a number of references cited in this topic.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
Список литературы
Основные статьи
Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the subcommittee on von Willebrand factor. J Thromb Haemost. 2006 Oct;4(10):2103-14.Полный текст Аннотация
Pavord S, Rayment R, Madan B, et al; on behalf of the Royal College of Obstetricians and Gynaecologists. Management of inherited bleeding disorders in pregnancy. Green-top guideline No 71 (joint with UKHCDO). BJOG. 2017 Jul;124(8):e193–263.Полный текст Аннотация
Tosetto A, Rodeghiero F, Castaman G, et al. A quantitative analysis of bleeding symptoms in type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1 vWD). J Thromb Haemost. 2006 Apr;4(4):766-73.Полный текст Аннотация
Keeling D, Tait C, Makris M. Guideline on the selection and use of therapeutic products to treat haemophilia and other hereditary bleeding disorders: a United Kingdom Haemophilia Center Doctors' Organisation (UKHCDO) guideline approved by the British Committee for Standards in Haematology. Haemophilia. 2008 Jul;14(4):671-84.Полный текст Аннотация
Статьи, указанные как источники
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Отличия
- Mild hemophilia A
- Inherited platelet function disorder
Больше ОтличияРекомендации
- Management of inherited bleeding disorders in pregnancy
- The diagnosis and management of von Willebrand disease
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