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

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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.

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