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 factorVIII 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 canresult from either a quantitative (types 1 and 3) or qualitative (type 2) defect in vWF. Type 2 vWD has been further classified into fourdistinct subtypes; 2A, 2B, 2M and 2N. The diagnosis of vWD requires attention to personal and family history of excessive bleeding andconfirmation by laboratory evaluation. A mild chronic thrombocytopenia is often seen in type 2B vWD. Patients with mild vWD oftenhave both a normal bleeding time and normal APTT. Specific tests for vWD diagnosis involve vWF antigen level, vWF activity (ristocetincofactor), and factor VIII activity. Once a diagnosis is established, additional tests that aid in classifying the type of vWD includeristocetin-induced platelet aggregation and vWF multimer analysis.
Published by | Perhimpunan Dokter Spesialis Patologi Klinik Indonesia |
Journal Name | Indonesian Journal of Clinical Pathology and Medical Laboratory (IJCPML) |
Contact Phone | +6285733220600 |
Contact Name | Dr. dr. Puspa Wardhani, SpPK |
Contact Email | admin@indonesianjournalofclinicalpathology.org |
Location | Kota adm. jakarta selatan, Dki jakarta INDONESIA |
Website | patologi| https://www.indonesianjournalofclinicalpathology.org/index.php/patologi| |
ISSN | ISSN : 08544263, EISSN : 24774685, DOI : -, |
Core Subject | Health, Science, |
Meta Subject | Biochemistry, Genetics & Molecular Biology, Health Professions, Medicine & Pharmacology, Neuroscience, |
Meta Desc | Indonesian Journal of Clinical Pathology and Medical Laboratory (IJCPML) is a journal published by “Association of Clinical Pathologist” professional association. This journal displays articles in the Clinical Pathology and Medical Laboratory scope. Clinical Pathology has a couple of subdivisions, namely: Clinical Chemistry, Hematology, Immunology and Serology, Microbiology and Infectious Disease, Hepatology, Cardiovascular, Endocrinology, Blood Transfusion, Nephrology, and Molecular Biology. Scientific articles of these topics, mainly emphasize on the laboratory examinations, pathophysiology, and pathogenesis in a disease. |
Penulis | Sindunata, R , Probohoesodo, M. Y. |
Publisher Article | Indonesian Association of Clinical Pathologist and Medical laboratory |
Subtitle Article | INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY Vol 13, No 1 (2006) |
Scholar Google | http://scholar.google.com/scholar?q=%2Bintitle%3A&… |
View Article | https://www.indonesianjournalo… |
DOI | https://doi.org/10.24293/ijcpml.v13… |
DOI Number | DOI: 10.24293/ijcpml.v13i1.895 |
Download Article [1] | https://www.indonesianjournalofclinicalp… |
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