Ageing patients with severe haemophilia: complications and comorbidities

Abstract

Background: Advances in the treatment of haemophilia patients have led to a significant increase in life expectancy. Diseases and conditions developing now were not previously encountered in this population group as patients were dying younger. Prevalence of haemophiliaassociated complications is increasing with age and modern diseases of ageing population are developing as well. Methods: The review included patients with severe haemophilia A or B and ≥ 40 years of age. Patient interview, physical examination and laboratory tests were performed. Results: In Slovenia, there are 35 patients with severe haemophilia, aged ≥ 40 years; 34 patients with haemophilia A and 1 patient with haemophilia B. Their mean age is 49.9 (range: 40–65) years. The hepatitis C virus infection rate is 100 %, in most cases chronic hepatitis. The HIV infection rate is low, 11.8 %. All patients sufferfrom arthropathy; 42.9 % receive prophylactic treatment. One patient (2.9 %) suffers from ischaemic heart disease. the prevalence of arterial hypertension is 38.2 %, of diabetes 2.9 %, of hypercholesterolemia 45.2 %; 21.2 % of patients are smokers. The mean body mass index is 25.3. Inhibitors developed in 2 of 34 patients. 23.5 % ofthe patients have university degree, 23.5 % have a full-time job and 57.1 % have family with children. The mean subjective quality-of-life score (1 to 10) is 6.83. Conclusions: Our data are comparable with other European countries. A high proportion of older haemophilia patients have secondary prophylaxis, and the usage has even increased recently. There is a strikingly low HIV infection prevalence due to treatment with local Slovenian plasma concentrate in the past and low presence of inhibitors. The number of haemophilia patients in Slovenia is too small to conclude that mutations in the factor VIII gene are reason for that. Cardiovascular risk factors do not differ in comparison to the general population.