Does haemophilia influence cancer‐related mortality in HIV‐negative patients?

Abstract

Clinical investigations and animal studies suggest haemophilia specific effects on cancer-related mortality aside from virus induced malignancies. Analysis of results in the literature proposes that coagulation factor deficiency might inhibit cancer metastasis through decreased activation of thrombin. On the other hand, substitution of coagulation factor might increase cancer rates. A review of epidemiological studies was conducted to survey the clinical data on cancer rates. Clinical investigations concerning cancer-related mortality in haemophilia always deal with virus-related malignancies caused by HIV and/or hepatitis C virus (HCV) infections. Therefore, analysis of cancer rates and standardized mortality ratios (SMR) of cancer in the literature was conducted under exclusion of HIV infection and concomitant malignancies like non-Hodgkin-lymphomas and under exclusion of HCV-related deaths caused by liver disease and hepatocellular carcinoma. The survey covers epidemiological studies which report causes of deaths of more than 8000 haemophilia patients, including more than 2700 HIV-negative patients. Results show virus independent cancer rates of 8-16% of deaths. Analysis of corresponding SMRs supports the hypothesis that cancer rates, unaffected through HIV or hepatoma, are decreased in haemophilia when compared with the general population. Prospective data collection regarding factor consumption as well as severity of haemophilia in virus negative cancer patients is needed to investigate the interaction between haemophilia and cancer.

Causes of Death Among 379 Patients With Hemophilia: A Developing Country’s Report

“…A survey on 717 Dutch patients with hemophilia has revealed that individuals with hemophilia have a protection against ischemic heart disease, whereas it has been reported there are more cancer-related mortalities. 20 There were 6 episodes of death due to various cancers, whereas in the current survey, there was only death due to heart problems. This may be relevant somehow to the fluctuation in the pattern of deaths among developed and developing countries.…”

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

“…In contrast with these findings, other studies have found a similar or even higher rate of non‐HIV/HCV‐related cancers in hemophiliacs than in non‐hemophiliacs [18,19]. In a recent review on epidemiological studies regarding cancer in hemophilia, Miesbach and Seifried [6] found that non‐HIV/HCV‐related cancers accounted for 8–16% of all deaths in hemophiliacs with a SMR lower than one in all studies, indicating a lower cancer mortality in the virally uninfected hemophilia population than in the matched general male population. The low SMR due to non‐HCC/HIV‐related cancers found in the present study (0.3) is in keeping with the majority of literature data and confirms the results from our previous study [2].…”

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.…”