Von Willebrand disease screening
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Von Willebrand disease Microchapters |
Diagnosis |
---|
Treatment |
Case Studies |
Von Willebrand disease screening On the Web |
American Roentgen Ray Society Images of Von Willebrand disease screening |
Risk calculators and risk factors for Von Willebrand disease screening |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
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 von Willebrand factor antigen and activity have been tried in the screening for von Willebrand disease.[1]
- 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.[2]
- Scoring key for the Pediatric Bleeding Questionnaire used in screening Von willebrand disease is shown below:[3]
- A score of 3 or more is highly predictive of vWD.[3]
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 |
References
- ↑ Verfaillie CJ, De Witte E, Devreese KM (2013). "Validation of a new panel of automated chemiluminescence assays for von Willebrand factor antigen and activity in the screening for von Willebrand disease". Int J Lab Hematol. 35 (5): 555–65. doi:10.1111/ijlh.12087. PMID 23551532.
- ↑ Naik S, Teruya J, Dietrich JE, Jariwala P, Soundar E, Venkateswaran L (2013). "Utility of platelet function analyzer as a screening tool for the diagnosis of von Willebrand disease in adolescents with menorrhagia". Pediatr Blood Cancer. 60 (7): 1184–7. doi:10.1002/pbc.24456. PMID 23335259.
- ↑ 3.0 3.1 Mittal N, Naridze R, James P, Shott S, Valentino LA (2015). "Utility of a Paediatric Bleeding Questionnaire as a screening tool for von Willebrand disease in apparently healthy children". Haemophilia. 21 (6): 806–11. doi:10.1111/hae.12689. PMC 4673659. PMID 25982122.