Von Willebrand disease screening: Difference between revisions
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{{Von Willebrand disease}} | {{Von Willebrand disease}} | ||
{{CMG}} | {{CMG}} {{AE}} {{PTD}} | ||
==Overview== | ==Overview== |
Revision as of 20:12, 28 December 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Prince Tano Djan, BSc, MBChB [2]
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.[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]
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.[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
- ↑ 1.0 1.1 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.
- ↑ 2.0 2.1 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.