UNIVERSITAS AIRLANGGA



Detail Article

Indonesian Journal of Clinical Pathology and Medical Laboratory

ISSN 0854-4263

Vol. 13 / No. 1 / Published : 2006-11

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Original Article :

Pathogenesis and diagnostics factors of von willebrand disease

Author :

  1. Rony Sindunata*1
  2. Maria Yolanda Probohoesodo*2
  1. Bagian Patologi Klinik Fakultas Kedokteran Universitas Airlangga – RSU Dr. Soetomo Surabaya
  2. Bagian Patologi Klinik Fakultas Kedokteran Universitas Airlangga – RSU Dr. Soetomo Surabaya

Abstract :

von Willebrand disease (vWD) is an autosomal inherited bleeding disorder caused by a deficiency or abnormality of von Willebrand factor (vWF). vWF is a large multimeric glycoprotein that mediates platelet adhesion at the site of vessel injury. It also protects factor VIII from proteolytic degradation in the circulation. vWD has a prevalence of about 1% in the general population but less than 10% have bleeding symptoms. Bleeding symptoms are usually mucocutaneous and post surgical with varying severity. This disorder can result from either a quantitative (types 1 and 3) or qualitative (type 2) defect in vWF. Type 2 vWD has been further classified into four distinct subtypes; 2A, 2B, 2M and 2N. The diagnosis of vWD requires attention to personal and family history of excessive bleeding and confirmation by laboratory evaluation. A mild chronic thrombocytopenia is often seen in type 2B vWD. Patients with mild vWD often have both a normal bleeding time and normal APTT. Specific tests for vWD diagnosis involve vWF antigen level, vWF activity (ristocetin cofactor), and factor VIII activity. Once a diagnosis is established, additional tests that aid in classifying the type of vWD include ristocetin-induced platelet aggregation and vWF multimer analysis.

Keyword :

Diagnostics factor, von Willebrand disease.,


References :

Kasper CK,(2005) von Willebrand disease : An Introductory Discussion for Young physicians; Available from: http://www.ckasper@laoh.ucla.edu. (accessed Oct 7, 2005) - : -

Castaman G,(2003) von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica ; 88 - : -

Biner B,(2005) von Willebrand factor and von Willebrand disease. Haema 8 (3) - : -

Federici AB,(2003) von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica ; 88 - : -

Rodeghiero F,(2003) von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica ; 88 - : -

Mannucci PM,(2003) von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica ; 88 -



Archive Article

Cover Media Content

Volume : 13 / No. : 1 / Pub. : 2006-11
  1. Levels Of B-hcg Among Patients With Hydatiform Mole Before And After Curettage
  2. Colony Count Candida Albicans Of Stool In Autism Spectrum Disorders
  3. The Comparison Of Wet Mount And Gram Stain Method For Vaginal Smear In Bacterial Vaginosis
  4. Microbial Patterns Based On Type Of Spesimens And Its Sensitivity To Antimicrobial Drugs
  5. The Value Of Sdldl Of Youngsters And Its Correlation With Other Lipids
  6. Lipid Profile In Type 2 Diabetic Mellitus Patient’s
  7. Pathogenesis And Diagnostics Factors Of Von Willebrand Disease
  8. Limited Systemic Sclerosis In A Young Boy
  9. The Correlation Between Fecal Occult Blood Test Using Anti-human Hemoglobin And Microscopic Examination
  10. Advantage And Disadvantage Of Quality Assurance Based On Point Of Care Testing/poct

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