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

Massimo Franchini; Caterina Di Perna; Cristina Santoro; Giancarlo Castaman; Simona Maria Siboni; Ezio Zanon; Silvia Linari; Paolo Gresele; Samantha Pasca; Antonio Coppola; Rita Santoro; Mariasanta Napolitano; Paola Ranalli; Annarita Tagliaferri; On Behalf of the Italian Association of Haemophilia Centres

doi:10.1055/s-0035-1564844

Seminars in Thrombosis and Hemostasis, Table of Contents

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

Authors

Massimo Franchini

¹Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy
Caterina Di Perna

²Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy
Cristina Santoro

³Department of Cellular Biotechnology and Hematology, Sapienza University of Rome, Rome, Italy
Giancarlo Castaman

⁴Haemophilia Centre of Vicenza, Vicenza, Italy

⁵Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Florence, Florence, Italy
Simona Maria Siboni

⁶Angelo Bianchi Bonomi Haemophilia and Thrombosis Centre, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico of Milan, Milan, Italy
Ezio Zanon

⁷Haemophilia Centre of Padova, Padova, Italy
Silvia Linari

⁵Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Florence, Florence, Italy
Paolo Gresele

⁸Haemophilia Centre of Perugia, Perugia, Italy
Samantha Pasca

⁹Haemophilia Centre of Udine, Udine, Italy
Antonio Coppola

¹⁰Haemophilia Centre of Naples, Naples, Italy
Rita Santoro

¹¹Regional Reference Centre for Inherited Bleeding and Thrombotic Disorders, Catanzaro, Italy
Mariasanta Napolitano

¹²Haemophilia Centre, Hematology Unit, University of Palermo, Palermo, Italy
Paola Ranalli

¹³Haemophilia Centre of Pescara, Pescara, Italy
Annarita Tagliaferri

²Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy

On Behalf of 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.

Keywords

von Willebrand disease - cancer - bleeding - mortality

Full Text

References

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