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

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, their management 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‐infected with 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 with mild/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.

Cancer detection and management in patients with haemophilia: a retrospective European multicentre study

“…In this study, no bleeding complications were observed in PWH undergoing chemotherapy or radiotherapy. This observation contrasts that of an Italian series involving 122 PWH, in which bleeding complications occurred more often in patients receiving chemotherapy (14%) or radiotherapy (19%) than in those undergoing invasive (8%) or surgical procedures (0.2%). Most notably, in this published series, the patients treated by chemotherapy or radiotherapy were not given clotting factor concentrate prophylaxis.…”

The Incidence Rates and Standardized Incidence Ratios of Cancer in Hemophilic HIV/AIDS Patients in Taiwan

“…14 A previous study by Rabkin et al in 1992 showed that with the exception of Kaposi's sarcoma and Non-Hodgkin's lymphoma, cancer incidence was not increased in hemophilic HIV-positive patients compared to the general population of the United States. 15 Similar results were also seen in an Italian study by Tagliaferri et al 16 Our study showed that hemophilic HIV-positive patients were also at increased risk of cancers of the oral cavity, oropharynx and hypopharynx, and larynx when compared to the general population of Taiwan.…”

LRP1/CD91 is up‐regulated in monocytes from patients with haemophilia A: a single‐centre analysis

“…As previously mentioned, the ubiquitous heat shock protein receptor LRP1/CD91 regulates signal transduction pathways and has been implicated in the pathogenesis of a number of human diseases. Thus, the LRP1/CD91 over-expression found in this study could help us to elucidate the molecular mechanisms underlying some of the still unexplained clinical findings recently observed in ageing haemophilia patients, such as the higher rates of hypertension and diabetes and lower mortality from ischaemic heart disease and cancers in comparison with the rates in the general, non-haemophilic population [22][23][24][25][26][27][28]. Indeed, a number of studies have documented that LRP1 is not only important for regulating vascular tone and smooth muscle cell proliferation, thus protecting from atherosclerosis [7,8], but it is also involved in the mechanisms of apoptosis and in the initiation of immune responses to tumours [29,30].…”