title: Cancers in Patients with von Willebrand Disease: A Survey from the Italian Association of Haemophilia Centres.
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.
authors.affiliation: Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Franchini
authors.affiliation: Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy.
authors.forename: Caterina
authors.initials: C
authors.lastname: Di Perna
authors.affiliation: Department of Cellular Biotechnology and Hematology, Sapienza University of Rome, Rome, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Santoro
authors.affiliation: Haemophilia Centre of Vicenza, Vicenza, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Castaman
authors.affiliation: Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico of Milan, Milan, Italy.
authors.forename: Simona Maria
authors.initials: SM
authors.lastname: Siboni
authors.affiliation: Haemophilia Centre of Padova, Padova, Italy.
authors.forename: Ezio
authors.initials: E
authors.lastname: Zanon
authors.affiliation: Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Florence, Florence, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Linari
authors.affiliation: Haemophilia Centre of Perugia, Perugia, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Gresele
authors.affiliation: Haemophilia Centre of Udine, Udine, Italy.
authors.forename: Samantha
authors.initials: S
authors.lastname: Pasca
authors.affiliation: Haemophilia Centre of Naples, Naples, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Coppola
authors.affiliation: Regional Reference Centre for Inherited Bleeding and Thrombotic Disorders, Catanzaro, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Santoro
authors.affiliation: Haemophilia Centre, Hematology Unit, University of Palermo, Palermo, Italy.
authors.forename: Mariasanta
authors.initials: M
authors.lastname: Napolitano
authors.affiliation: Haemophilia Centre of Pescara, Pescara, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Ranalli
authors.affiliation: Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy.
authors.forename: Annarita
authors.initials: A
authors.lastname: Tagliaferri
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D006782:Hospitals, Special; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008875:Middle Aged; D009369:Neoplasms / Q000401:mortality / Q000628:therapy; D011446:Prospective Studies; D011795:Surveys and Questionnaires; D014842:von Willebrand Diseases / Q000150:complications / Q000401:mortality / Q000628:therapy