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

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 HIV related. 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.