OBJECTIVES:

The ultra-orphan acquired hemophilia A (AHA) is a bleeding disorder induced by autoantibodies against coagulation factor VIII. AHA causes potentially very severe trauma-induced or spontaneous bleeding. Awareness measurements by professional societies, but also pharmaceutical industry increased awareness of the disease in Germany. Here we examine trends in frequency of AHA diagnosis and treatment based on German hospital billing data.

METHODS:

Reports from the German DRG institute (InEK) and Federal Bureau of Statistics (DESTATIS) were analyzed for the period of 2010 to 2021 using Microsoft Access® 2019.

RESULTS: Cases coded as main or secondary diagnosis of AHA (D68.31, ICD10-GM) increased from 215 per year (2010) to 633 (2015; +132%) and slowly decreased again to 447 (2021). Changes in the use of factor products from 2019 to 2021 included: activated prothrombin complex concentrate (APCC, FEIBA®) from 12% to 7%; recombinant factor VIIa (rFVIIa, NovoSeven®) from 48% to 44%; recombinant factor VIII (rFVIII, human or porcine) from 22% to 24%; plasma-derived factor VIII 17.5% to 14.7%; emicizumab 0% to 9.4%. Comorbidity and complication levels (PCCL) and length of hospital stay differed with treatments (means): APCC, PCCL 3.1, length of stay 31 days; rFVIIa: 2.5, 29 days; rVIII: 2.1, 21 days; pVIII 1.8, 30 days; emicizumab: 2.1, 34 days.

CONCLUSIONS: The success of awareness campaigns increased the number of hospital cases with AHA in Germany. The number of cases per year has remained >400 since 2015. The use of emicizumab increased to about 10% of cases, whereas other treatments have changed only slightly since 2019. Length of hospital stay and complexity of cases vary by treatment modality. These findings warrant further investigation in the national registry