Von Willebrand disease differential diagnosis: Difference between revisions

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__NOTOC__
__NOTOC__
{{Von Willebrand disease}}
[[Image:Home_logo1.png|right|250px|link=https://www.wikidoc.org/index.php/Von_Willebrand_disease]]
{{CMG}}  {{AE}} {{PTD}}
{{CMG}}  {{AE}} {{PTD}}


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[[vWD]] must be differentiated from platelet disorders, thrombophilias, and hemophilias as shown below:
[[vWD]] must be differentiated from platelet disorders, thrombophilias, and hemophilias as shown below:
{| class="wikitable"
!Variable
!vWD
!Thrombophilia
!Hemophilia
!Platelet disorder
|-
|Genetics
|Mostly autosomal dominant and rarely recessive
|Mostly by mutation in the ''F5'' gene (at position 1691) and prothrombin G20210A
|X-linked recessive 
|Variable depending of type
|-
|Presentation
|Children mostly present with symptoms of bruising and epistaxis.<ref name="pmid26375306">{{cite journal| author=Sanders YV, Fijnvandraat K, Boender J, Mauser-Bunschoten EP, van der Bom JG, de Meris J et al.| title=Bleeding spectrum in children with moderate or severe von Willebrand disease: Relevance of pediatric-specific bleeding. | journal=Am J Hematol | year= 2015 | volume= 90 | issue= 12 | pages= 1142-8 | pmid=26375306 | doi=10.1002/ajh.24195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26375306  }}</ref>Adults present with bleeding after surgery, mucosa-associated bleeding, [[menorrhagia|heavy menstrual periods]] and [[postpartum hemorrhage|postpartum hemorrhage]]. Severe [[internal bleeding|internal]] or [[hemarthrosis|joint bleeding]] is rare (which only occurs in type 3 vWD). <ref name="pmid22918553">{{cite journal| author=de Wee EM, Sanders YV, Mauser-Bunschoten EP, van der Bom JG, Degenaar-Dujardin ME, Eikenboom J et al.| title=Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease. | journal=Thromb Haemost | year= 2012 | volume= 108 | issue= 4 | pages= 683-92 | pmid=22918553 | doi=10.1160/TH12-04-0244 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22918553  }}</ref>
|Manifests as deep vein thrombosis or pulmonary embolism with sudden-onset shortness of breath, chest pain, palpitations
|Deep tissue bleeding into joint spaces
|Superficial bleeding
|-
|Platelet count
|Normal
|Normal
|Normal
|Low
|-
|Clotting factor 8 or 9
|Low to normal factor 8
|factor IX is increase<ref name="pmid19846852">{{cite journal| author=Simioni P, Tormene D, Tognin G, Gavasso S, Bulato C, Iacobelli NP et al.| title=X-linked thrombophilia with a mutant factor IX (factor IX Padua). | journal=N Engl J Med | year= 2009 | volume= 361 | issue= 17 | pages= 1671-5 | pmid=19846852 | doi=10.1056/NEJMoa0904377 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19846852  }}</ref>
|Decreased factor VIII or IX
|Normal
|-
|vWF
|Dcreased
|Increased<ref name="pmid20231535">{{cite journal| author=Smith NL, Chen MH, Dehghan A, Strachan DP, Basu S, Soranzo N et al.| title=Novel associations of multiple genetic loci with plasma levels of factor VII, factor VIII, and von Willebrand factor: The CHARGE (Cohorts for Heart and Aging Research in Genome Epidemiology) Consortium. | journal=Circulation | year= 2010 | volume= 121 | issue= 12 | pages= 1382-92 | pmid=20231535 | doi=10.1161/CIRCULATIONAHA.109.869156 | pmc=2861278 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20231535  }}</ref>
|Normal
|Normal
|-
|Prothrobin time
|Normal
|Normal
|Normal
|Normal
|-
|aPTT
|Prolonged
|Normal
|Increased
|Normal
|-
|Treatment
|Desmopressin or vWF or factor VIII concentrates
|Variable depending on the cause
|Factor VIII or IX concentrate
|Depends on type
|}
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}

Latest revision as of 21:03, 26 February 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Prince Tano Djan, BSc, MBChB [2]

Overview

vWD must be differentiated from platelet disorders, thrombophilias, and hemophilias based on genetic disoder, clinical presentation, laboratory findings and treatment.

Von Willebrand disease differential diagnosis

vWD must be differentiated from platelet disorders, thrombophilias, and hemophilias as shown below:

References

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