SMEJKAL, Petr, J. J. MICHIELS, Ondřej ZAPLETAL, Jan BLATNÝ, A. BATOROVA, T. PRICANGOVA, U. BUDDE, C. HERMANS, K. MAYGER, G. MOORE, I. VANGENEGTEN and Miroslav PENKA. European Clinical, Laboratory and Molecular (2020 ECLM) Diagnostic Work-Up and Classification of Von Willebrand Disease from the Perspectives of Clinicians and Scientists. Thrombosis & Haemostasis: Research. Austin Publishing, 2019, vol. 3, No 3, p. 1-15. ISSN 2689-9663.
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Basic information
Original name European Clinical, Laboratory and Molecular (2020 ECLM) Diagnostic Work-Up and Classification of Von Willebrand Disease from the Perspectives of Clinicians and Scientists
Authors SMEJKAL, Petr (203 Czech Republic, guarantor, belonging to the institution), J. J. MICHIELS (528 Netherlands), Ondřej ZAPLETAL (203 Czech Republic, belonging to the institution), Jan BLATNÝ (203 Czech Republic, belonging to the institution), A. BATOROVA (703 Slovakia), T. PRICANGOVA (703 Slovakia), U. BUDDE (276 Germany), C. HERMANS (56 Belgium), K. MAYGER (826 United Kingdom of Great Britain and Northern Ireland), G. MOORE (826 United Kingdom of Great Britain and Northern Ireland), I. VANGENEGTEN (56 Belgium) and Miroslav PENKA (203 Czech Republic, belonging to the institution).
Edition Thrombosis & Haemostasis: Research, Austin Publishing, 2019, 2689-9663.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30205 Hematology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
WWW URL
RIV identification code RIV/00216224:14110/19:00115715
Organization unit Faculty of Medicine
Keywords in English Von willebrand disease; Von willebrand factor VWF antigen; VWF collagen binding; VWF ristocetine cofactor; VWF multimers; and VWF propeptide; VWF ristocetine induced platelet aggregation; Autosomal recessive; Autosomal dominant; Heredity
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 2/6/2020 07:31.
Abstract
The International Society of Thrombosis Haemostasis (ISTH) classification separated Von Willebrand Disease (VWD) into type 1 and type 2 by the use of four insensitive Von Willebrand Factor (VWF) assays Ristocetine Co-factor (VWF:RCo), VWF Antigen (VWF:Ag), Ristocetine Induced Platelet Agglutination (RIPA) and VWF multimers in a low resolution gel. A complete set of VWF parameters is mandatory to discriminate between all variants of VWD type 1, 2 and 3 and includes Bleeding Time (BT), PFA-100 closure times, FVIII:C, VWF:RCo activity, VWF Collagen Binding (VWF:CB), RIPA, VWF propeptide (VWF:pp), multimeric analysis of VWF and the response of FVIII:C and VWF parameters to DDAVP. We here translate the ISTH into European, Clinical, Laboratory and Molecular (2020 ECLM) classification of the Von Willebrand Disease (VWD) related to the domain location of the Molecular (M) efect in the VWF gene to detect all variants of VWD.
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