Von Willebrand disease screening

Von Willebrand disease Microchapters
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Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Von Willebrand disease from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
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Other Imaging Findings
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Case #1
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Risk calculators and risk factors for Von Willebrand disease screening

Von Willebrand disease Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Von Willebrand disease from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
Chest X Ray
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Von Willebrand disease screening On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Von Willebrand disease screening All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Von Willebrand disease screening
on Von Willebrand disease screening
Von Willebrand disease screening in the news
Blogs on Von Willebrand disease screening
Directions to Hospitals Treating Von Willebrand disease
Risk calculators and risk factors for Von Willebrand disease screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

There is no established screening modality for Von Willebrand disease. However, several questionnaires and laboratory investigations have been developed to help in the screening process. Von Willebrand factor antigen and activity have been tried in the screening for von Willebrand disease. The use of platelet function analyzer as a screening tool for the diagnosis of von Willebrand disease in adolescents with menorrhagia is not sufficiently sensitive to detect Von Willebrand Ristocetin cofactor activity (RCoF) values <50 IU/dl with normal multimer patterns.

Screening

• There is no established screening modality for Von Willebrand disease. However, several questionnaires and laboratory investigations have been developed to help in the screening process.
• The von Willebrand factor antigen and activity have been tried in the screening for von Willebrand disease.

• The use of platelet function analyzer as a screening tool for the diagnosis of von Willebrand disease in adolescents with menorrhagia is not sufficiently sensitive to detect Von Willebrand Ristocetin cofactor activity (RCoF) values <50 IU/dl with normal multimer patterns.
• Scoring key for the Pediatric Bleeding Questionnaire used in screening Von willebrand disease is shown below:
• A score of 3 or more is highly predictive of vWD.

Symptom/score	-1	0	1	2	3	4
Epistaxis	-	No or trivial (≤5 per year)	>5 per year OR >10 minutes duration	Consultation only	Packing, cauterization or antifibrinolytics	Blood transfusion, replacement therapy or desmopressin
Cutaneous	-	No or trivial (≤1cm)	>1cm AND no trauma	Consultation only	-	-
Minor wounds	-	No or trivial (≤5 per year)	>5 per year OR >5 minutes duration	Consultation only or Steristrips	Surgical hemostasis or antifibrinolytics	Blood transfusion, replacement therapy or desmopressin
Oral cavity	-	No	Reported at least once	Consultation only	Surgical hemostasis or antifibrinolytics	Blood transfusion, replacement therapy or desmopressin
Gastrointestinal tract	-	No	Identified cause	Consultation or spontaneous	Surgical hemostasis, antifibrinolytics, blood transfusion, replacement therapy or desmopressin	-
Tooth extraction	No bleeding in at least 2 extractions	None done or no bleeding in 1 extraction	Reported, no consultation	Consultation only	Resuturing, repacking or antifibrinolytic	Blood transfusion, replacement therapy or desmopressin
Surgery	No bleeding in at least 2 surgeries	None done or no bleeding in 1	Reported, no consultation	Consultation only	Surgical hemostasis or antifibrinolytics	Blood transfusion, replacement therapy or desmopressin
Menorrhagia	-	No	Reported or consultation only	Antifibrinolytics or contraceptive pill use	D&C or iron therapy	Blood transfusion, replacement therapy or desmopressin
Post-partum	No bleeding in at least 2 deliveries	No deliveries or no bleeding in 1 delivery	Reported or consultation only	D&C, iron therapy or antifibrinolytics	Blood transfusion, replacement therapy or desmopressin	-
Muscle hematoma	-	Never	Post-trauma, no therapy	Spontaneous, no therapy	Spontaneous or traumatic, requiring replacement therapy or desmopressin	Spontaneous or traumatic, requiring surgical intervention or blood transfusion
Hemarthrosis	-	Never	Post-trauma, no therapy	Spontaneous, no therapy	Spontaneous or traumatic, requiring replacement therapy or desmopressin	Spontaneous or traumatic, requiring surgical intervention or blood transfusion
Central nervous system	-	Never	-	-	Subdural, any intervention	Intracerebral, any intervention
Other (eg. umbilical stump bleeding, cephalhematoma, post-circumcision bleeding, post-venipuncture bleeding, and macroscopic hematuria)	-	No	Reported	Consultation only	Surgical hemostasis, antifibrinolytics or iron therapy	Blood transfusion, replacement therapy or desmopressin