Summary. Background: The increased life expectancy of the hemophilia population, primarily as a result of advances in factor replacement therapy, has enabled hemophiliacs to reach an older age. Consequently, age-related diseases, such as cardiovascular disorders and cancers, are being increasingly recognized in such patients. However, only few data are available on such co-morbidities, theirmanagement and impact on the primary bleeding disorders. Objectives: With the aim of investigating several still unclear issues regarding cancers in hemophilia patients, we conducted, on behalf the Italian Association of Hemophilia Centers (AICE), a study on cancers among Italian hemophiliacs. Patients: Data pertaining to 122 hemophiliacs with 127 cancers between 1980 and 2010 were retrospectively collected in 21 centers of the AICE which chose to participate. Results: Sixty-nine percent of cancers were recorded during the decade 2001–2010. Eighty-three percent of patients were infected with hepatitis C virus (HCV) and 22% of them were also co-infectedwith human immunodeficiency virus (HIV). Forty-three percent of cancers were HCV-related, whereas 9%were HIVrelated. Virus-related cancers were more frequent and non-virus-related cancers less frequent in patients with severe hemophilia than in those withmild/moderate forms (P = 0.0004). The non-virus-related standardized mortality ratio (SMR) was 0.3. Hemorrhagic complications occurred more frequently in patients undergoing chemotherapy (14%) or radiotherapy (19%). Conclusions: The results of the present study confirm that cancers have become a new challenge for physicians working in hemophilia centers and underline the need for prospective trials to better assess the epidemiology and to optimize the management of hemophiliacs with cancer.

Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers / Tagliaferri, A; Di Perna, C; Santoro, Cristina; Schinco, P; Santoro, R; Rossetti, G; Coppola, A; Morfini, M; Franchini, M; Italian Association of Hemophilia, Centers; Mazzucconi, Maria Gabriella; Castaman, G; Scaraggi, Fa; Macchi, S; Pasca, S; Valdrè, L; Zanon, E; Radossi, P; Serino, Ml; Cantori, I; Napolitano, M; Lapecorella, M; Giuffrida, Ac; Dragani, A; Delios, G; Arbasi, M. C.. - In: JOURNAL OF THROMBOSIS AND HAEMOSTASIS. - ISSN 1538-7933. - STAMPA. - 10 (1)(2012), pp. 90-95. [10.1111/j.1538-7836.2011.04566.x]

Cancers in patients with hemophilia: a retrospective study from the Italian Association of Hemophilia Centers.

SANTORO, Cristina;MAZZUCCONI, Maria Gabriella;
2012

Abstract

Summary. Background: The increased life expectancy of the hemophilia population, primarily as a result of advances in factor replacement therapy, has enabled hemophiliacs to reach an older age. Consequently, age-related diseases, such as cardiovascular disorders and cancers, are being increasingly recognized in such patients. However, only few data are available on such co-morbidities, theirmanagement and impact on the primary bleeding disorders. Objectives: With the aim of investigating several still unclear issues regarding cancers in hemophilia patients, we conducted, on behalf the Italian Association of Hemophilia Centers (AICE), a study on cancers among Italian hemophiliacs. Patients: Data pertaining to 122 hemophiliacs with 127 cancers between 1980 and 2010 were retrospectively collected in 21 centers of the AICE which chose to participate. Results: Sixty-nine percent of cancers were recorded during the decade 2001–2010. Eighty-three percent of patients were infected with hepatitis C virus (HCV) and 22% of them were also co-infectedwith human immunodeficiency virus (HIV). Forty-three percent of cancers were HCV-related, whereas 9%were HIVrelated. Virus-related cancers were more frequent and non-virus-related cancers less frequent in patients with severe hemophilia than in those withmild/moderate forms (P = 0.0004). The non-virus-related standardized mortality ratio (SMR) was 0.3. Hemorrhagic complications occurred more frequently in patients undergoing chemotherapy (14%) or radiotherapy (19%). Conclusions: The results of the present study confirm that cancers have become a new challenge for physicians working in hemophilia centers and underline the need for prospective trials to better assess the epidemiology and to optimize the management of hemophiliacs with cancer.
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11573/462736
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