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Titolo:
Appropriate laboratory assessment as a critical facet in the proper diagnosis and classification of von Willebrand disorder
Autore:
Favaloro, EJ;
Indirizzi:
Westmead Hosp, Diagnost Haemostasis Lab, Dept Haematol,Inst Clin Pathol & Med Res, Western Sydney Area Hlth Serv, Westmead, NSW 2145, Australia Westmead Hosp Westmead NSW Australia 2145 , Westmead, NSW 2145, Australia
Titolo Testata:
BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY
fascicolo: 2, volume: 14, anno: 2001,
pagine: 299 - 319
SICI:
1521-6926(200106)14:2<299:ALAAAC>2.0.ZU;2-Z
Fonte:
ISI
Lingua:
ENG
Soggetto:
PLATELET-FUNCTION ANALYZER; COLLAGEN-BINDING ASSAY; HIGH-SHEAR STRESS; FACTOR-VIII; VONWILLEBRAND-FACTOR; MONOCLONAL-ANTIBODY; PRIMARY HEMOSTASIS; VWD SUBTYPES; RISTOCETIN COFACTOR; DESMOPRESSIN DDAVP;
Keywords:
von Willebrand factor; VWF; von Willebrand disease; von Willebrand disorder; VWD; laboratory assessment; diagnosis; review;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
83
Recensione:
Indirizzi per estratti:
Indirizzo: Favaloro, EJ Westmead Hosp, Diagnost Haemostasis Lab, Dept Haematol,Inst Clin Pathol & Med Res, Western Sydney Area Hlth Serv, Westmead, NSW 2145, Australia Westmead Hosp Westmead NSW Australia 2145 SW 2145, Australia
Citazione:
E.J. Favaloro, "Appropriate laboratory assessment as a critical facet in the proper diagnosis and classification of von Willebrand disorder", BEST P R C, 14(2), 2001, pp. 299-319

Abstract

The correct diagnosis and classification of von Willebrand disease or disorder (VWD) is crucial because the presenting biological activity of von Willebrand factor (VWF) determines both the haemorrhagic risk and the subsequent clinical management. A variety of laboratory assays may be employed, notnecessarily restricted to assessments of VWF. Because of assay limitationsand von Willebrand disease heterogeneity, no single test procedure is sufficiently 'robust' to permit the detection of all VWD variants. Classically,the test panel might include any combination of: (a) skin bleeding time, (b) von Willebrand factor antigen assay, (c) factor VIII C level, (d) assessment of 'functional' von Willebrand factor (collagen-binding activity or ristocetin co-factor assay), (e) ristocetin-induced platelet aggregation, and(f) multimer analysis. There have also been many new diagnostic developments that have begun to influence the diagnostic process. These include the automation of existing assay procedures, new automated platelet function analysers such as the PFA-100 (R), and specific von Willebrand factor-factor VIII-binding assays. This chapter focuses on the recommended laboratory process for the investigation of VWD. The selection of an appropriate combination test panel and testing sequence is crucial for the proper diagnosis and classification of congenital von Willebrand disease.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 17/01/21 alle ore 06:36:24