Von Willebrand factor and von Willebrand disease
Küçük Resim Yok
Tarih
2005
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Von Willebrand disease (vWD) is an autosomally 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. Also, it protects factor VIII from proteolytic degradation in the circulation. vWD has a prevalence of about 1% in the general population but less than 10% of them have bleeding symptoms. Bleeding symptoms are usually mucocutaneous and postsurgical 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. If the patient has a chronic blood loss, there may be accompanying iron deficiency anaemia and a mild chronic thrombocytopenia is often seen with type 2B vWD. Patients with mild vWD often have both a normal bleeding time and normal aPTT. Specific tests for vWD diagnose 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. There are two main options available for the treatment of bleeding episodes: desmopressin and replacement therapy with plasma-derived factor VIII/vWF concentrates. Antifibrinolytic agents and topical haemostatic preparations are also used as adjunctive therapies. Copyright © Hellenic Society of Haematology.
Açıklama
Anahtar Kelimeler
Desmopressin; Von Willebrand Disease; Von Willebrand Factor, Aminocaproic Acid; Antifibrinolytic Agent; Blood Clotting Factor 8; Desmopressin; Dna; Estrogen; Fibrin; Fibrin Glue; Glycoprotein; Hemostatic Agent; Iron; Messenger Rna; Metalloproteinase; Nonsteroid Antiinflammatory Agent; Plasminogen; Proteinase; Ristocetin; Signal Peptide; Thrombin; Tranexamic Acid; Von Willebrand Factor; Von Willebrand Factor Cleaving Proteinase; Analyzer; Bleeding; Bleeding Time; Clinical Trial; Cryoprecipitate; Dimerization; Enzyme Linked Immunosorbent Assay; Flushing; Gene; Gene Deletion; Glycosylation; Headache; Human; Hyponatremia; Iron Deficiency Anemia; Nausea; Nucleotide Sequence; Partial Thromboplastin Time; Phenotype; Protein Degradation; Protein Structure; Protein Synthesis; Review; Seizure; Substitution Therapy; Tachycardia; Thrombocyte Adhesion; Thrombocyte Aggregation; Thrombocyte Count; Thrombosis; Von Willebrand Disease
Kaynak
HAEMA
WoS Q Değeri
Scopus Q Değeri
N/A
Cilt
8
Sayı
3
