Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series

Acquired hemophilia A (AHA) is a rare disease that requires urgent management. Currently, there is no consensus regarding optimal management in aged people. This systematic review aimed to describe diagnosis, clinical features, management, and endpoints in population aged 65 years or over with AHA. A literature search up to and including 31 May 2017 was performed in Medline, Embase, and Scopus. The search strategy on article titles comprised the following terms: "acquired" AND ("hemophilia A" OR "haemophilia A"). Filters were applied for age (65 years or older), publication type (case reports and case series), and studies including human beings only. There was no language restriction in the search strategy. Studies with no data on immunosuppressive therapy, and studies in other languages than English or French were excluded. Patient-level and study-level information was extracted. In total, 270 studies were identified by the literature search. After exclusion of duplicates, and studies presenting exclusion criteria, 80 articles including 159 cases were included in the final review. These 159 cases were 76.1 ± 7.2 years old, and were mainly men (64%). There is wide variety in the therapies used to eradicate the Factor VIII autoantibody, and efficacy is difficult to assess. The majority of patients with AHA receive immunosuppressants. Mortality is high, and likely depends on the rapidity of diagnosis and implementation of adequate management and monitoring.