This information has been fed into a database which now has >30 years cumulated clinical experience with which we can evaluate epidemiological factors such changes in disease status, morbidity and mortality. It is interesting that this database precedes the HIV/AIDS epidemic and therefore provides an opportunity to assess its impact on the epidemiological characteristics of the haemophiliac population. RAD001 clinical trial The survey was not compulsory and so the number of responses each year varied, and on average approximately two-thirds of clinics replied in any given year. In 2009/2010 the database included information from almost 10 000 patients with bleeding disorders: ∼600

with haemophilia B, ∼4000 with haemophilia A and ∼5000 with von Willebrand disease. The factor activity and inhibitor

responder rates for patients with haemophilia A and B in the periods 2009/2010 and 2008/2009 are shown in Table 4. One question we sought to answer using the database was whether anti-PD-1 antibody inhibitor the prevalence of inhibitors was increasing following the introduction of a number of recombinant products. Interestingly, between the years 2000 and 2010 we observed a marked decrease in the proportion of patients with inhibitors, and this may be explained in part by the better immune tolerability of newer recombinant products. With regard to von Willebrand disease there were almost 5000 patients on the database in 2009/2010, but less than 20% (861 of 4995) of these had ristocetin cofactor activity levels ≤30% [38]. The HIV/AIDS epidemic had a catastrophic impact on the haemophiliac population, but we appear to have overcome the worst of it, and in the 2009/2010 cohort 372 (4.1%) haemophiliac patients were HIV positive and there were only six deaths which were AIDS related. This compares with thousands of haemophiliac patients who were

HIV positive in the 1990s. The improvement in the clinical picture in relation to HIV/AIDS is reflected by a significantly reduced death rate resulting from HIV over the last 15 years (Fig. 3). When we compare results for the periods selleckchem 1982–1995 to 1996–2010 there was an approximate 15-fold reduction in deaths related to HIV in the haemophiliac population. Over the period 1978–2010 the incidence of liver disease has varied widely. However, the trend has been for liver disease to increase in the German haemophilic population and this is particularly noticeable when the pre-1996 period is compared with the post 1996 period. Overall, there was almost a twofold increase in liver disease post 1996 (P < 0.002). Up to 1998 the number of deaths due to cancer per year was very low with only isolated cases reported. However, since 1998 the rate of reporting of malignancy-related deaths has increased sharply (Fig. 4). This is due to an increase in the number of hepatocellular carcinomas which, in turn, is related to the increased number of patients that are hepatitis C positive.