OBJECTIVES: Hemophilia B (HB), also called factor IX (FIX) deficiency, is a genetic disorder caused by missing or defective FIX. Although it is passed down from parents to children, about 1/3 of cases are caused by spontaneous mutations. Birth prevalence is around one in 20,000 male births. Main treatment are concentrated FIX-products of plasmatic or recombinant origin. Since 2008 the Paul-Ehrlich-Institute (PEI) publishes annual epidemiological report. We have taken this further by examining frequency of in-hospital HB cases and inhospital use of FIX.

METHODS: Data from the German DRG-Institute (InEK) 2005-2016, Statistical Office (DESTATIS) 2005-2016 and hospital quality reports for 2015 were analyzed for cases of HB and FIX-treatment. Statistical Analysis with Microsoft-Excel and Access version 2016.

RESULTS: Hospital cases with main diagnosis (MD) of HB decreased from 70 (2005) to 51 (2015, -27%). cases with secondary diagnosis (SD) remained stable, around 450 per year; and 449 cases in 2015. Patient number in the German Haemophilia Registry (DHR) increased from 537 in 2008 to 700 in 2014. Mean age of hospitalized patients (30.3 ± 23.9 years) and gender distribution (95% male) remained stable over time. Length of stay did not differ between age groups. The decrease in cases was not associated with lower in-Hospital FIX usage; constantly around 5m units annually or 8.6% of all FIX used in Germany. There was no age pattern for the selection of plasmatic or recombinant FIX, but a hospital pattern. There was a growing Preference for use recombinant FIX (rFIX in 2015: 44%) over plasmatic FIX.

CONCLUSIONS: The rate of hospitalization of patients with HB decreased overall slightly in the last decade. Treatment shifted to the ambulatory sector. The use of FIX in hospital remained stable due to a trend for much higher use per case. The in-hospital share of recombinant FIX is increasing versus plasmatic FIX.