European Clinical, Laboratory and Molecular (2020 ECLM)
Diagnostic Work-Up and Classification of Von Willebrand Disease
from the Perspectives of Clinicians and Scientists

J 2019

European Clinical, Laboratory and Molecular (2020 ECLM) Diagnostic Work-Up and Classification of Von Willebrand Disease from the Perspectives of Clinicians and Scientists

SMEJKAL, Petr, J. J. MICHIELS, Ondřej ZAPLETAL, Jan BLATNÝ, A. BATOROVA et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30205 Hematology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

V originále

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.

Citovat

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.

@article{1659559,
   author = {Smejkal, Petr and Michiels, J. J. and Zapletal, Ondřej and Blatný, Jan and Batorova, A. and Pricangova, T. and Budde, U. and Hermans, C. and Mayger, K. and Moore, G. and Vangenegten, I. and Penka, Miroslav},
   article_number = {3},
   keywords = {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},
   language = {eng},
   issn = {2689-9663},
   journal = {Thrombosis & Haemostasis: Research},
   title = {European Clinical, Laboratory and Molecular (2020 ECLM) Diagnostic Work-Up and Classification of Von Willebrand Disease from the Perspectives of Clinicians and Scientists},
   url = {https://austinpublishinggroup.com/thrombosis-haemostasis/all-issues.php},
   volume = {3},
   year = {2019}
}

TY  - JOUR
ID  - 1659559
AU  - Smejkal, Petr - Michiels, J. J. - Zapletal, Ondřej - Blatný, Jan - Batorova, A. - Pricangova, T. - Budde, U. - Hermans, C. - Mayger, K. - Moore, G. - Vangenegten, I. - Penka, Miroslav
PY  - 2019
TI  - European Clinical, Laboratory and Molecular (2020 ECLM) Diagnostic Work-Up and Classification of Von Willebrand Disease from the Perspectives of Clinicians and Scientists
JF  - Thrombosis & Haemostasis: Research
VL  - 3
IS  - 3
SP  - 1-15
EP  - 1-15
PB  - Austin Publishing
SN  - 26899663
KW  - Von willebrand disease
KW  - Von willebrand factor VWF antigen
KW  - VWF collagen binding
KW  - VWF ristocetine cofactor
KW  - VWF multimers
KW  - and VWF propeptide
KW  - VWF ristocetine induced platelet aggregation
KW  - Autosomal recessive
KW  - Autosomal dominant
KW  - Heredity
UR  - https://austinpublishinggroup.com/thrombosis-haemostasis/all-issues.php
L2  - https://austinpublishinggroup.com/thrombosis-haemostasis/all-issues.php
N2  - 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.
ER  -

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. \textit{Thrombosis \&{} Haemostasis: Research}. Austin Publishing, 2019, vol.~3, No~3, p.~1-15. ISSN~2689-9663.

Detailed Information on Publication Record