|
Title
|
|
|
|
PFA-100 monitoring of von Willebrand factor (VWF) responses to desmopressin (DDAVP) and factor VIII/VWF concentrate substitution in von Willebrand disease type 1 and 2
|
|
|
Author
|
|
|
|
|
|
|
Abstract
|
|
|
|
| Dose-response relationship was studied between PFA-100 closure times (PFA CTs) and factor (F)VIII-von Willebrand factor (VWF) parameters in patients with von Willebrand disease (VWD) type 1 and type 2 before and after treatment with DDAVP (n=84) or FVIII/VWF concentrate (n=38). DDAVP treatment of patients with VWD type I normalised the PFA CTs by increasing VWF levels to normal. Of the 14 patients with VWD type 2, PFA CTs did not normalize in eight. Haemate-P substitution in patients with VWD type I induced a less favourable response as compared to DDAVP, because PFA CTs did not correct in all patients. Of 12 patients with VWD type 2 treated with Haemate-P, six showed a correction of PFA CTs (<250 sec), which correlated with the normalisation of the VWF CB/Ag ratio. In-vitro studies were performed by using whole blood of patients with VWD and adding various amounts of FVIII/VWF concentrate. Addition of Haemate-P induced an increase of the VWF CB/Ag ratio from 0.30 to 0.70 in blood of patients with VWD type 2 with correction of the PFA CTs. Immunate did not result in an increase of VWF CB/Ag ratio in blood of VWD type 2 patients, and the PFA CTs remained prolonged. We conclude that PFA-100 might be an adequate instrument not only for diagnosis but also for monitoring of DDAVP responses and FVIII/VWF substitution of patients with VWD type 1 and 2, but this is dependent upon the type of VWD and the concentrate used. |
|
|
|
Language
|
|
|
|
English
|
|
|
Source (journal)
|
|
|
|
Thrombosis and haemostasis : international journal for vascular biology and medicine. - Stuttgart, 1976
|
|
|
Publication
|
|
|
|
Stuttgart
:
Schattauer
,
2008
|
|
|
ISSN
|
|
|
|
0340-6245
[print]
2567-689X
[online]
|
|
|
DOI
|
|
|
|
10.1160/TH07-08-0527
|
|
|
Volume/pages
|
|
|
|
100
:3
(2008)
, p. 462-468
|
|
|
ISI
|
|
|
|
000259229100018
|
|
|
Full text (Publisher's DOI)
|
|
|
|
|
|
|
Full text (publisher's version - intranet only)
|
|
|
|
|
|