Abstract
Diagnostics and treatment of severe forms of haemorrhagic diatheses (HD), such as haemophilia A, B or type 3 von Willebrand disease (VWD) need high standards in haemophilia treatment centers (HTCs). Due to their generally low incidence, a close cooperation of haemophilia treaters is needed to optimize treatment strategies. For this purpose, the “Kompetenznetz Hämorrhagische Diathesen Ost” was founded. The first project was the conduction of a survey of epidemiological data of patients with HA, HB and type 3 VWD in all HTCs in the eastern part of Germany. The study assessed the data regarding numbers of patients treated with HA, HB or type 3 VWD, disease severity, regime of coagulation factor replacement (CFR) and the frequency of factor VIII and IX inhibitors. Data of 838 patients (a prevalence of 52.38 per 1 million citizen) from 27 HTCs were evaluated (5–151 per HTCs). Among the included patients, 201 (prevalence: 12.56/million) were children or adolescents (< 18 years). 81 children suffered from severe HA (prevalence: 5.06/million), 20 from severe HB (prevalence: 1.25/million) and 10 from type 3 VWD (prevalence: 0.63/million). In 637 adults (prevalence: 39.82/million) we found 246 with severe HA (prevalence: 15.38/million), 51 severe HB (prevalence: 3.19/million) and 28 VWD of type 3 (prevalence: 1.75/million). The most commonly used treatment of CFR in patients with severe disease was prophylactic modality. 90% of the children received prophylaxis, but also in 64% of the adults an intermittent or longterm secondary prophylaxis was the preferred treatment. At the time of survey conduction, a factor VIII or IX inhibitor was present in 1.9% of the children and in 1.6% of the adults with haemophilia. Our data allow an overview of the number of patients and treatment strategies in the eastern part of Germany.
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