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Semin Thromb Hemost 2016; 42(01): 036-041
DOI: 10.1055/s-0035-1564844
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Cancers in Patients with von Willebrand Disease: A Survey from the Italian Association of Haemophilia Centres

Massimo Franchini
1   Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy
,
Caterina Di Perna
2   Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy
,
Cristina Santoro
3   Department of Cellular Biotechnology and Hematology, Sapienza University of Rome, Rome, Italy
,
Giancarlo Castaman
4   Haemophilia Centre of Vicenza, Vicenza, Italy
5   Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Florence, Florence, Italy
,
Simona Maria Siboni
6   Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico of Milan, Milan, Italy
,
Ezio Zanon
7   Haemophilia Centre of Padova, Padova, Italy
,
Silvia Linari
5   Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Florence, Florence, Italy
,
Paolo Gresele
8   Haemophilia Centre of Perugia, Perugia, Italy
,
Samantha Pasca
9   Haemophilia Centre of Udine, Udine, Italy
,
Antonio Coppola
10   Haemophilia Centre of Naples, Naples, Italy
,
Rita Santoro
11   Regional Reference Centre for Inherited Bleeding and Thrombotic Disorders, Catanzaro, Italy
,
Mariasanta Napolitano
12   Haemophilia Centre, Hematology Unit, University of Palermo, Palermo, Italy
,
Paola Ranalli
13   Haemophilia Centre of Pescara, Pescara, Italy
,
Annarita Tagliaferri
2   Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy
,
On Behalf of the Italian Association of Haemophilia Centres › Author Affiliations
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Publication History

Publication Date:
23 November 2015 (online)

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Abstract

Besides its essential role in hemostasis, there is growing evidence that von Willebrand factor (VWF) has an additional antitumor effect. To elucidate the clinical significance of this biological activity we conducted a retrospective study on cancers among Italian patients with von Willebrand disease (VWD) on behalf of the Italian Association of Haemophilia Centres (AICE). A questionnaire to collect demographic, clinical, and treatment data of VWD patients with cancer was sent to all the 54 Italian Haemophilia Treatment Centres (HTCs) members of AICE. Overall, 18 HTCs (33%) provided information on 92 VWD patients (61 alive and 31 deceased) with 106 cancers collected during the period 1981 to 2014. Of them, 19 (18%) were hematological cancers and 87 (82%) were solid cancers. A total of 61% of patients had type 1, 36% type 2 (12% type 2A, 14% type 2B, 9% type 2M, and 1% type 2N), and 3% type 3 VWD: this distribution was significantly different from that observed in the whole VWD population (79% type 1, 16% type 2 [8% type 2A, 4% type 2B, 2% type 2M, 2% type 2N], and 5% type 3; type 2 vs. non-type 2: p < 0.001). Overall, VWD patients with cancer underwent 52 invasive and 72 surgical procedures, were treated with VWF/factor VIII (FVIII) concentrates in 77 cases, with desmopressin (DDAVP) alone in 24 cases and with DDAVP and VWF/FVIII concentrates in 7 cases. Hemorrhagic complications were observed only rarely (2% of invasive procedures and radiotherapy and 6% of surgical interventions). The data collected by this survey document that a substantial number of cancers are recorded among VWD patients and that these patients are safely managed by HTC physicians through a multidisciplinary approach.

Keywords

von Willebrand disease - cancer - bleeding - mortality

* The complete list of coauthors for the study (Italian Association of Haemophilia Centres, with the site of the Haemophilia Centre given in parentheses, is as follows.


Cosimo Ettorre and Renato Marino (Bari); Piergiorgio Iannaccaro (Catanzaro); Flora Peyvandi (Milano); Giovanni Di Minno (Napoli); Gianna Franca Rivolta and Gabriele Quintavalle (Parma); Alfredo Dragani (Pescara); Silvia Macchi (Ravenna); Gianluca Sottilotta (Reggio Calabria); Attilia Maria Pizzini (Reggio Emilia); Maria Gabriella Mazzucconi (Roma); Piercarla Schinco (Torino); Anna Chiara Giuffrida (Verona).


 

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