FAKTOR PATOGENESIS DAN DIAGNOSIS PENYAKIT von Willebrand
DOI:
https://doi.org/10.24293/ijcpml.v13i1.895Keywords:
Diagnostics factor, von Willebrand disease.Abstract
von Willebrand disease (vWD) is an autosomal inherited bleeding disorder caused by a deficiency or abnormality of von Willebrandfactor (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.
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Submitted
2018-03-15
Accepted
2018-03-15
Published
2018-03-15
How to Cite
[1]
Sindunata, R. and Probohoesodo, M.Y. 2018. FAKTOR PATOGENESIS DAN DIAGNOSIS PENYAKIT von Willebrand. INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY. 13, 1 (Mar. 2018), 23–20. DOI:https://doi.org/10.24293/ijcpml.v13i1.895.
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