PMID- 29913537
OWN - NLM
STAT- MEDLINE
DCOM- 20190219
LR  - 20190320
IS  - 1098-9064 (Electronic)
IS  - 0094-6176 (Linking)
VI  - 45
IP  - 1
DP  - 2019 Feb
TI  - Evaluation of the Utility of von Willebrand Factor Propeptide in the Differential 
      Diagnosis of von Willebrand Disease and Acquired von Willebrand Syndrome.
PG  - 36-42
LID - 10.1055/s-0038-1660481 [doi]
AB  - An increased von Willebrand factor propeptide (VWFpp) to VWF antigen (VWF:Ag) 
      ratio (VWFpp/VWF:Ag) indicates an enhanced clearance of VWF. This finding has 
      been described in von Willebrand disease (VWD) and in acquired von Willebrand 
      syndrome (AVWS). A distinction between these two diseases, one congenital and the 
      other acquired, is primarily based on family and personal history of bleeding. 
      However, if this information is scanty, the diagnosis might be challenging due to 
      the lack of an effective diagnostic biomarker. In this cross-sectional study, we 
      assessed the ability of VWFpp/VWF:Ag for the differential diagnosis between VWD 
      and AVWS. VWFpp/VWF:Ag was measured in a group of 153 patients (125 with VWD and 
      28 with AVWS). Most patients with AVWS and VWD showed an increased VWFpp/VWF:Ag, 
      although to variable degrees. A marked increase of VWFpp/VWF:Ag was mainly 
      associated with the diagnosis of AVWS and VWD type 1 Vicenza. A receiver 
      operating characteristic curve was used to identify the optimal cutoff of 
      VWFpp/VWF:Ag for discrimination of patients with a modestly increased (most VWD 
      cases) versus those with a markedly increased clearance (AVWS and VWD type 1 
      Vicenza), and this cutoff was identified at the value of 3.9 (sensitivity: 0.70, 
      specificity: 0.97). The ROC curve sorting from a logistic model containing 
      VWFpp/VWF:Ag, age, and sex had an area under the curve (AUC) of 0.88 (95% 
      confidence interval: 0.80-0.95). A subsequent molecular evaluation discriminated 
      VWD type 1 Vicenza from AVWS. In conclusion, VWFpp/VWF:Ag appears helpful to 
      discriminate patients with a markedly increase VWF clearance (AVWS or VWD type 1 
      Vicenza) from those with a modestly increased clearance (most VWD patients).
CI  - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
FAU - Stufano, Francesca
AU  - Stufano F
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
FAU - Boscarino, Marco
AU  - Boscarino M
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
FAU - Bucciarelli, Paolo
AU  - Bucciarelli P
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
FAU - Baronciani, Luciano
AU  - Baronciani L
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
FAU - Maino, Alberto
AU  - Maino A
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
AD  - Internal Medicine Unit, Azienda Provinciale per i Servizi Sanitari, Trento, 
      Italy.
FAU - Cozzi, Giovanna
AU  - Cozzi G
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
FAU - Peyvandi, Flora
AU  - Peyvandi F
AD  - Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca' 
      Granda Ospedale Maggiore Policlinico and Luigi Villa Foundation, Milan, Italy.
AD  - Department of Pathophysiology and Transplantation, Universita degli Studi di 
      Milano, Milan, Italy.
LA  - eng
PT  - Journal Article
DEP - 20180618
PL  - United States
TA  - Semin Thromb Hemost
JT  - Seminars in thrombosis and hemostasis
JID - 0431155
RN  - 0 (von Willebrand Factor)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Diagnosis, Differential
MH  - Female
MH  - Humans
MH  - Male
MH  - Young Adult
MH  - von Willebrand Diseases/*diagnosis/pathology
MH  - von Willebrand Factor/*metabolism
COIS- F.P. has received honoraria for participating as a speaker at satellite symposia 
      and educational meetings organized by Ablynx, Grifols, Sobi, Shire, F. 
      Hoffmann-La Roche, and Alnylam. She is recipient of research grant funding from 
      Ablynx, Novo Nordisk, Kedrion, and Biokit paid to Fondazione Luigi Villa, and she 
      has received consulting fees from Kedrion and LFB. She is also member of the 
      scientific advisory boards of Ablynx, F. Hoffmann-La Roche, and Shire.The other 
      authors had no interests which might be perceived as posing a conflict or bias.
EDAT- 2018/06/19 06:00
MHDA- 2019/03/21 06:00
CRDT- 2018/06/19 06:00
PHST- 2018/06/19 06:00 [pubmed]
PHST- 2019/03/21 06:00 [medline]
PHST- 2018/06/19 06:00 [entrez]
AID - 10.1055/s-0038-1660481 [doi]
PST - ppublish
SO  - Semin Thromb Hemost. 2019 Feb;45(1):36-42. doi: 10.1055/s-0038-1660481. Epub 2018 
      Jun 18.