Management of acquired hemophilia A: results from the Spanish registry

Abstract

The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.

Graphical abstract

Figure 1.

Outcomes according to immunosuppressive therapy. *<0.05 vs STR (Fisher’s exact test). Missing data were not considered. In the Kaplan-Meier curves of panel B, tick marks indicate patients whose data were censored by the time of last follow-up date. CFX, cyclophosphamide; CNI, calcineurin inhibitors; RTX, rituximab; STR, steroids.

Figure 2.

Survival according to selected clinical characteristics. Kaplan-Meier survival curves according to age (≥65 vs < 65 y) (A) and achievement of CR (yes vs no) (B), were generated, and compared by log-rank tests. Tick marks indicate patients whose data were censored by the time of last follow-up date.