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| __NOTOC__
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| {{Von Willebrand disease}}
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| __NOTOC__ | | __NOTOC__ |
| {{Von Willebrand disease}} | | {{Von Willebrand disease}} |
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| {{CMG}} | | {{CMG}} {{AE}} {{PTD}} |
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| ==Overview== | | ==Overview== |
| | The ISTH-Bleeding Assessment Tool is a validated instrument that is used to screen patients referred for bleeding symptoms for further laboratory testing. The three main screening tests used in the diagnosis of VWD include vonWillebrand Factor (VWF) antigen, platelet-dependent VWF activity, and factor VIII activity. |
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| ==Screening== | | ==Screening== |
| *There is no established screening modality of Von Willebrand disease. However, several questionnaires and laboratory investigations have been developed to help in the screening process.
| | The ISTH-Bleeding Assessment Tool is a validated instrument that is used to screen patients referred for bleeding symptoms for further laboratory testing.<ref name="pmid30064913">{{cite journal |vauthors=Rao ES, Ng CJ |title=Current approaches to diagnostic testing in von Willebrand Disease |journal=Transfus. Apher. Sci. |volume= |issue= |pages= |date=July 2018 |pmid=30064913 |doi=10.1016/j.transci.2018.07.005 |url=}}</ref> |
| *The von Willebrand factor antigen and activity have been tried in the screening for von Willebrand disease.<ref name="pmid23551532">{{cite journal |vauthors=Verfaillie CJ, De Witte E, Devreese KM |title=Validation of a new panel of automated chemiluminescence assays for von Willebrand factor antigen and activity in the screening for von Willebrand disease |journal=Int J Lab Hematol |volume=35 |issue=5 |pages=555–65 |year=2013 |pmid=23551532 |doi=10.1111/ijlh.12087 |url=}}</ref>
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| *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.<ref name="pmid23335259">{{cite journal |vauthors=Naik S, Teruya J, Dietrich JE, Jariwala P, Soundar E, Venkateswaran L |title=Utility of platelet function analyzer as a screening tool for the diagnosis of von Willebrand disease in adolescents with menorrhagia |journal=Pediatr Blood Cancer |volume=60 |issue=7 |pages=1184–7 |year=2013 |pmid=23335259 |doi=10.1002/pbc.24456 |url=}}</ref>
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| *Scoring key for the Pediatric Bleeding Questionnaire used in screening Von willebrand disease is shown below:<ref name="pmid25982122">{{cite journal |vauthors=Mittal N, Naridze R, James P, Shott S, Valentino LA |title=Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children |journal=Haemophilia |volume=21 |issue=6 |pages=806–11 |year=2015 |pmid=25982122 |pmc=4673659 |doi=10.1111/hae.12689 |url=}}</ref>
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| A score of 3 or more is highly predictive of vWD.<ref name="pmid25982122">{{cite journal |vauthors=Mittal N, Naridze R, James P, Shott S, Valentino LA |title=Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children |journal=Haemophilia |volume=21 |issue=6 |pages=806–11 |year=2015 |pmid=25982122 |pmc=4673659 |doi=10.1111/hae.12689 |url=}}</ref>
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| {| class="wikitable"
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| !Symptom/score
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| !-1
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| !0
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| !1
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| !2
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| !3
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| !4
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| |-
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| !'''Epistaxis'''
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| |'''-'''
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| |No or trivial (≤5 per
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| year)
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| |>5 per year OR >10
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| minutes duration
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| |Consultation only
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| |Packing, cauterization or
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| antifibrinolytics
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Cutaneous'''
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| |'''-'''
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| |No or trivial (≤1cm)
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| |>1cm AND no trauma
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| |Consultation only
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| | -
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| | -
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| |-
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| !'''Minor wounds'''
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| |'''-'''
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| |No or trivial (≤5 per
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| year)
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| |>5 per year OR >5
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| minutes duration
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| |Consultation only or Steristrips
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| |Surgical hemostasis or
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| antifibrinolytics
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Oral cavity'''
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| |'''-'''
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| |No
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| |Reported at least once
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| |Consultation only
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| |Surgical hemostasis or
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| antifibrinolytics
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Gastrointestinal'''
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| '''tract'''
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| |'''-'''
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| |No
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| |Identified cause
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| |Consultation or spontaneous
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| |Surgical hemostasis,
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| antifibrinolytics, blood
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| transfusion, replacement therapy
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| or desmopressin
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| | -
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| |-
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| !'''Tooth extraction'''
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| |No bleeding in at least
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| 2 extractions
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| |None done or no
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| bleeding in 1
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| extraction
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| |Reported, no
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| consultation
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| |Consultation only
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| |Resuturing, repacking or
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| antifibrinolytic
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Surgery'''
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| |No bleeding in at least
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| 2 surgeries
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| |None done or no
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| bleeding in 1
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| |Reported, no
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| consultation
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| |Consultation only
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| |Surgical hemostasis or
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| antifibrinolytics
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Menorrhagia'''
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| | -
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| |No
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| |Reported or consultation
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| only
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| |Antifibrinolytics or
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| contraceptive pill use
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| |D&C or iron therapy
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |-
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| !'''Post-partum'''
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| |No bleeding in at least
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| 2 deliveries
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| |No deliveries or no
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| bleeding in 1 delivery
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| |Reported or consultation
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| only
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| |D&C, iron therapy or
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| antifibrinolytics
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| | -
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| |-
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| !'''Muscle hematoma'''
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| | -
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| |Never
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| |Post-trauma, no therapy
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| |Spontaneous, no therapy
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| |Spontaneous or traumatic,
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| requiring replacement therapy or
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| desmopressin
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| |Spontaneous or traumatic,
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| requiring surgical intervention
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| or blood transfusion
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| |-
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| !'''Hemarthrosis'''
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| | -
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| |Never
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| |Post-trauma, no therapy
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| |Spontaneous, no therapy
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| |Spontaneous or traumatic,
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| requiring replacement therapy or
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| desmopressin
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| |Spontaneous or traumatic,
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| requiring surgical intervention
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| or blood transfusion
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| |-
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| !'''Central nervous'''
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| '''system'''
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| | -
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| |Never
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| | -
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| | -
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| |Subdural, any intervention
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| |Intracerebral, any intervention
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| |-
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| !'''Other'''
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| '''(eg. umbilical stump bleeding,'''
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| '''cephalhematoma,'''
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| '''post-circumcision bleeding, post-venipuncture bleeding,'''
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| '''and macroscopic hematuria)'''
| | The three main screening tests used in the diagnosis of VWD include: |
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| | * VonWillebrand Factor (VWF) antigen |
| |No
| | * Platelet-dependent VWF activity |
| |Reported
| | * Factor VIII activity. |
| |Consultation only
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| |Surgical hemostasis,
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| antifibrinolytics or iron therapy
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| |Blood transfusion, replacement
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| therapy or desmopressin
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| |}
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| ==References== | | ==References== |