Acquired Haemophilia A: A 15-Year Single-Centre Experience of Demography, Clinical Features and Outcome

Abstract

Acquired haemophilia A (AHA) is a rare severe bleeding disorder resulting from the production of autoantibodies directed against coagulation factor VIII. At presentation, bleeding events can be severe, and an early diagnosis and treatment are of major importance. The current study aims to analyse the treated patients who have been diagnosed with AHA for a better understanding of our population and treatment outcome. We conducted a retrospective study with 26 patients who had been diagnosed with AHA and who were treated in our hospital between January 2006 and January 2021. The patients ranged in age from 30 to 85 years old: 46.10% were men, 46.10% had no known underlying condition, 27% had an underlying malignancy, 7.60% presented with other diseases: psoriatic arthritis and Paget's disease, and 19.30% presented with AHA during puerperium. All of the patients had bleeding events and were treated with bypass agents for this as well as with immunosuppressive therapy to eradicate the inhibitor. A total of 53.80% of the patients had major bleeding. Sixty-nine percent of the patients achieved complete remission, but 26.90% died during the follow-up, although bleeding was not the cause of death in any of these cases. Our observations underline the importance of clinical suspicion and early referral to centres with experience and laboratory facilities for managing AHA.

Keywords: acquired; demography; haemophilia A; presentation.

Figure 1

Distribution of patients diagnosed with acquired haemophilia A by age range and sex.

Figure 2

Distribution of patients diagnosed with acquired haemophilia A by underlying condition and age. Independent samples from non-parametric tests: Kruskal–Wallis. “None” is also described as idiopathic in the text. “Other” includes psoriatic arthritis and Paget’s disease. * Isolated case.

Figure 3

(A) Major bleeding episodes according to FVIII levels. (B) Major bleeding episodes according to inhibitor level. * Isolated case. Two independent samples from the non-parametric tests: U Mann–Whitney.

Figure 4

Kaplan–Meier plot showing the overall survival of patients with acquired haemophilia A.

Figure 5

(A) Kaplan–Meier plot showing the overall survival according to age group. (B) Kaplan–Meier plot showing the overall survival according to transfusion requirements.

Figure 6

Overall survival according to underlying disease.

Figure 7

Overall survival according to major bleeding.