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RESEARCH PRODUCT
Cancers in Patients with von Willebrand Disease: A Survey from the Italian Association of Haemophilia Centres
Franchini MassimoDi Perna CaterinaSantoro CristinaCastaman GiancarloSiboni Simona MariaZanon EzioLinari SilviaGresele PaoloSamantha PascaAntonio CoppolaSantoro RitaMariasanta NapolitanoRanalli PaolaTagliaferri AnnaritaGiuffrida Anna Chiarasubject
Adultcongenital hereditary and neonatal diseases and abnormalitiesPediatricsmedicine.medical_specialtySettore MED/09 - Medicina InternaPopulationbleeding; cancer; mortality; von Willebrand disease; Hematology; Cardiology and Cardiovascular MedicineHaemophiliaHospitals Specialbleeding; cancer; mortality; von Willebrand disease; Adult; Aged; Aged 80 and over; Humans; Italy; Middle Aged; Prospective Studies; Hospitals Special; Neoplasms; Surveys and Questionnaires; von Willebrand Diseases; Hematology; Cardiology and Cardiovascular MedicineVon Willebrand factorNeoplasmsSurveys and Questionnaireshemic and lymphatic diseases80 and overmedicineVon Willebrand diseaseHumanscancerProspective StudiesDesmopressinProspective cohort studyeducationAgedAged 80 and overeducation.field_of_studySpecialbiologybusiness.industryRetrospective cohort studyHematologyMiddle Agedmedicine.diseasebleedingmortalityHospitalsbleeding; cancer; mortality; von Willebrand disease; Adult; Aged; Aged 80 and over; Humans; Italy; Middle Aged; Prospective Studies; Hospitals Special; Neoplasms; Surveys and Questionnaires; von Willebrand Diseasesvon Willebrand DiseasesItalyHemostasisbiology.proteinbusinessvon Willebrand diseaseCardiology and Cardiovascular Medicinemedicine.drugdescription
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.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2015-01-01 |