Franchini Massimo, Di Perna Caterina, Santoro Cristina, Castaman Giancarlo, Siboni Simona Maria, Zanon Ezio, Linari Silvia, Gresele Paolo, Pasca Samantha, Coppola Antonio, Santoro Rita, Napolitano Mariasanta, Ranalli Paola, Tagliaferri Annarita
Department of Haematology and Transfusion Medicine, Carlo Poma Hospital, Mantova, Italy.
Regional Reference Centre for Inherited Bleeding Disorders, University Hospital of Parma, Parma, Italy.
Semin Thromb Hemost. 2016 Feb;42(1):36-41. doi: 10.1055/s-0035-1564844. Epub 2015 Nov 23.
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.
除了在止血中发挥重要作用外，越来越多的证据表明血管性血友病因子（VWF）具有额外的抗肿瘤作用。为了阐明这种生物学活性的临床意义，我们代表意大利血友病中心协会（AICE）对意大利血管性血友病（VWD）患者中的癌症情况进行了一项回顾性研究。向AICE的所有54个意大利血友病治疗中心（HTC）成员发送了一份问卷，以收集患有癌症的VWD患者的人口统计学、临床和治疗数据。总体而言，18个HTC（33%）提供了1981年至2014年期间92例VWD癌症患者（61例存活，31例死亡）的106种癌症信息。其中，19种（18%）为血液系统癌症，87种（82%）为实体癌。共有61%的患者为1型VWD，36%为2型（12%为2A型，14%为2B型，9%为2M型，1%为2N型），3%为3型VWD：这种分布与整个VWD人群中观察到的分布有显著差异（79%为1型，16%为2型[8%为2A型，4%为2B型，2%为2M型，2%为2N型]，5%为3型；2型与非2型：p < 0.001）。总体而言，患有癌症的VWD患者接受了52次侵入性手术和72次外科手术，77例患者接受了VWF/凝血因子VIII（FVIII）浓缩物治疗，24例仅接受去氨加压素（DDAVP）治疗，7例接受DDAVP和VWF/FVIII浓缩物治疗。出血并发症很少见（侵入性手术和放疗的2%以及外科手术的6%）。本次调查收集的数据表明，VWD患者中记录了大量癌症病例，并且HTC的医生通过多学科方法对这些患者进行了安全管理。
