Acquired hemophilia: insights from a multicenter real-life study in a middle-income country

Abstract

Background Acquired hemophilia A (AHA) is a rare hemorrhagic disease with scarce published information from developing countries. Objectives The main objective was to describe the clinical manifestation and treatment, as well as laboratory characteristics of patients with AHA. Patients/methods We conducted a multicenter retrospective cohort study that included consecutive adult patients with AHA between 1999 and 2020 from Argentine hospitals. A comparison was made between the group achieving complete remission and the nonremission group. Logistic regression analysis was conducted to assess factors associated with death. Results The study included 68 patients. Nineteen hospitals from 11 cities were involved. The median follow-up was 12.5 months [interquartile range (IQR) 4–36]. Sixty-six patients (97%) had spontaneous bleeding at diagnosis (66% major bleeding). The median inhibitor titer was 27 (IQR 13–98) Bethesda units/ml (BU/ml). Hemostatic therapy was employed in 52 cases (67% utilized bypassing agents). The most used therapy was corticosteroids plus cyclophosphamide (40%). Complete remission was achieved in 45 patients (66%), with a median of 6 weeks (IQR 3–12). No differences were found in achieving complete remission with FVIII or inhibitor titer, nor in therapies. Nineteen patients died (28%). Eleven of the deaths were related to AHA or their treatments. The main cause of death was bleeding ( n = 8). Seven out of eight deaths due to bleeding occurred within 72 h. Conclusion We present the most extensive AHA study in Latin America. Unlike studies from other regions, we found a high rate of bleeding-related deaths. It may be necessary to improve early suspicion and diagnosis and enhance therapeutic management in our area.