Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels-Reference-Cited by-同舟云学术

Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels

Published:2017-11-23 Issue:21 Volume:130 Page:2344-2353
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Lavin Michelle¹²^ORCID,Aguila Sonia²,Schneppenheim Sonja³,Dalton Niall²,Jones Kenneth L.⁴,O’Sullivan Jamie M.²,O’Connell Niamh M.¹,Ryan Kevin¹,White Barry¹,Byrne Mary¹,Rafferty Marie¹,Doyle Mairead M.¹,Nolan Margaret¹,Preston Roger J. S.⁵,Budde Ulrich³,James Paula⁶,Di Paola Jorge⁴,O’Donnell James S.¹²

Affiliation:

1. National Coagulation Centre, St James’s Hospital, Dublin, Ireland;

2. Haemostasis Research Group, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland;

3. MEDILYS Laborgesellschaft mbH, ASKLEPIOS Klinik Altona, Zentrales Labor, Hamburg, Germany;

4. Department of Pediatrics and Human Genetics and Genomics Program, University of Colorado, Anschutz Medical Campus, Aurora, Colorado;

5. Department of Molecular and Cellular Therapeutics, Irish Centre for Vascular Biology, Royal College of Surgeons in Ireland, Dublin, Ireland; and

6. Department of Medicine, Queen's University, Kingston, ON, Canada

Abstract

Key Points Patients registered with low VWF have significant bleeding phenotypes that cannot be explained by concomitant bleeding disorders. Low VWF levels in the range of 30 to 50 IU/dL are predominantly due to reduced VWF synthesis/secretion rather than enhanced clearance.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/130/21/2344/1403351/blood786699.pdf

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