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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}} |