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2018 ; 16
(4
): 371-381
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Current challenges in the diagnosis and management of patients with inherited von
Willebrand s disease in Italy: an Expert Meeting Report on the diagnosis and
surgical and secondary long-term prophylaxis
#MMPMID29313798
Schinco P
; Castaman G
; Coppola A
; Cultrera D
; Ettorre C
; Giuffrida AC
; Marchesini E
; Marino R
; Milan M
; Molinari C
; Siboni SM
; Zanon E
; Federici AB
Blood Transfus
2018[Jul]; 16
(4
): 371-381
PMID29313798
show ga
Recent advances in the care of von Willebrand's disease (vWD) have allowed the
majority of patients to be managed adequately. Even in the more severe forms, it
is now possible to control recurrent bleeding through secondary long-term
prophylaxis with von Willebrand factor-containing concentrates. Moreover, in the
setting of surgical prophylaxis, the combination of interdisciplinary management
and close patient monitoring yields a positive outcome in nearly all cases,
although safety concerns remain. In clinical practice, the effectiveness of
therapy is hindered by the difficulties in making a rapid, yet accurate
diagnosis, in identifying the subgroup of bleeders who may benefit most from a
specific strategy, and in selecting the optimal product and regimen.Since
specific guidelines for heavy bleeders requiring short- and long-term prophylaxis
are still lacking, sharing the experience of experts dealing with vWD patients on
a daily basis is crucial to fill gaps in information relating to patient
management. To address this important issue, 13 Italian haematologists met in
Milan on April, 2, 2016 and in Florence on July, 9, 2016. A 30-question survey
constituted the input to discuss (i) optimisation of the diagnostic workflow for
vWD, (ii) the characteristics of patients who may benefit from secondary
long-term prophylaxis (in particular with the purified von Willebrand factor
concentrate with a low content of factor VIII), (iii) the key elements to
consider when selecting a concentrate and (iv) the pre-operative and
post-operative management of vWD patients. A summary of the main points covered
is provided in this report.