Haemophilia in Poland

Abstract

The primary aim of this study was to analyse the data on 2269 haemophilic patients in the Polish National Register of Inherited Bleeding Disorders -- 1953 haemophilia A patients and 316 haemophilia B patients. Haemophilia A occurred in 1512 families, haemophilia B in 240 families. In the majority of haemophilia A and B cases severe haemophilia prevailed (59.7% and 56.6% respectively). In about 50% of haemophilic patients, no family history of bleeding diathesis was reported. For haemophilia A patients the mean age was 30.9 years and for haemophilia B patients, 29.2 years. Prevalence of haemophilia in Poland is approximately 1:12 300 inhabitants (1:5600 males). The second aim was to describe the orthopaedic status of severe haemophilia patients and to relate this status to the type of replacement therapy they received prior to the study. Ninety-two severe haemophilia patients (median age 26.0 years) were enrolled in the study. Right and left knee, elbow and ankle joints were evaluated clinically using the Gilbert scale. X-ray examinations were evaluated according to the Pettersson scale. Knee joints proved to be most affected. Eighty-four patients (91.3%) reported pain. Only one scored 0 on the Gilbert scale, another on the Pettersson scale. Thirty-seven per cent of patients used orthopaedic equipment, either occasionally or constantly. Twenty-five per cent had a history of orthopaedic surgery. Thirty-eight per cent were unemployed with some form of social subvention. On-demand treatment was applied. None of the patients received primary prophylaxis. The mean consumption of clotting factor concentrates was 68 054 IU per patient during the 12 months prior to the current study. These results indicate that in Poland all severe haemophilia patients above 20 years are affected by haemophilic arthropathy.

Web‐enabled registry of inherited bleeding disorders in Portugal: conditions and perception of the patients

“…The results of these 168 responses confirmed the high prevalence of the disease in HA (75%) compared with HB (11.3%), in a similar ratio as observed in Spain and Poland [18,19]. The distribution of patients by severity is also similar to that reported from Spain, Canada and Sweden [18], confirming a large incidence in the severe levels or the existence of people with mild severity without diagnosis and treatment.…”

“…The observed ratio of HA to HB was 6.6:1, similar to the reported in studies from Spain (6.5:1) [18] and Poland (6.2:1) [19]. Moreover, the distribution of patients by severity is very similar to the reported values from Spain, Canada or Sweden [18].…”

Co-Infection of the Hepatitis C Virus With Other Blood-Borne and Hepatotropic Viruses Among Hemophilia Patients in Poland

“…The prevalence of hemophilia in Poland is approximately 1:12,300 inhabitants (13). The analysis presented in this study was part of a wider study assessing the staging of chronic liver disease in Polish HCV-infected hemophilic patients (HemC).…”

Physical and psychosocial challenges in adult hemophilia patients with inhibitors

“…Patients with inhibitors have worse treatment-related outcomes, including greater incidence of joint abnormalities, more rapid progression of arthropathy, more chronic joint pain,2–5 and an increased incidence of intracranial hemorrhage than patients without inhibitors 6. The assumption has been that these poor outcomes are the result of inadequately controlled intra-articular bleeding in patients with inhibitors.…”