Trend in Hospital Cases of Acquired Hemophilia A (AHA) 2010 - 2014 in Germany

S. Wahler1, A. Tiede2 (1Hamburg, Germany, 2Hannover, Germany)

Acquired problems and alterations of coagulation
Date: 17.02.2017,
Time: 17:15 - 18:15

Objective: Objective: Acquired hemophilia A (AHA) is a rare bleeding disorder, caused by the development of autoantibodies against human coagulation factor VIII. AHA may lead to spontaneous or trauma induced bleeds that are treated with bypassing agents. The incidence of the disease was estimated to about 1.4 per million per year in the UK. Data from Germany are not available. A specific ICD 10 code for AHA has existed since 2010, allowing epidemiological insights. We examined the frequency of AHA based on these data and compared it to data from the recent GTH-AH 01/2010 study.

Methods: Methods: The reports from German DRG Institute (InEK), Statistical Office (DESTATIS) and the hospital quality reports for 2010-2014 were analyzed for cases of AHA and treatments with high amounts of bypassing agents (APCC > 150.000 units, rFVIIa > 500 mg). Statistical analysis was performed using Microsoft-Excel and Access version 2013.

Results: Results: The number of cases with a main diagnosis of AHA (D68.31, ICD10-GM) increased from 29 (2010), over 40 (2011), 37 (2012), 69 (2013) to 109 (2014, + 275%). The mean age of patients (73.4 +/- 15.9 years) and the gender distribution (58% male) remained stable over time and were very similar to data from the GTH-AH study. The average length of hospital stay of male patients was with 25.2 days significantly longer than for females (18.4 days), possibly reflecting the trend towards a less favorable prognosis of AHA in male patients as seen in the GTH study. The number of cases with a secondary diagnosis of AHA increased moderately from 186 (2010) over 200 (2011), 128 (2012), 134 (2013) to 225 (2014, +21%). The total number of cases in 2014 was 334 (~4 per mio. per year), indicating a higher incidence than suggested from UK data. The increase in cases was not associated with a growth in treatments with high doses of rFVIIa (108 in 2010, 97 in 2014) or APCC (66 in 2010, 56 in 2014).

Conclusion: Conclusion: We found an increase in documented hospital cases with AHA from 2010 to 2014. The overall number exceeds the expected number of patients based on previously reported incidence. This may reflect a growing awareness towards AHA, under-diagnosis in previous studies, or both. Remarkably, the number of patients intensively treated with bypassing agents decreased, suggesting that higher awareness may lead to earlier diagnosis and prevention of high costs due to bleeding.