Background:

Acquired haemophilia A (AHA) is a rare severe condition that occurs in people with variable age. Neutralizing autoantibodies are formed against factor VIII (FVIII), causing spontaneous or trauma-induced bleeding. Immunosuppressive therapy (IST) results in remission in most patients despite high morbidity and mortality.

Aims:

Long-term follow-up data from the haemophilia centre of Rio de Janeiro (HEMORIO) were analysed to assess baseline characteristics and prognostic factors in patients with AHA.

Methods:

This is a retrospective cohort study in which all patients with laboratory confirmed AHA admitted to HEMORIO, the regional public blood center located in Rio de Janeiro, Brazil, were included. AHA was defined as FVIII inhibitor titres (IT) ≥ 0.6 Bethesda Units (BU) and levels of FVIII < 50 UI/dL. Complete remission (CR) was defined as FVIII inhibitor titres < 0.6 BU. Major bleeding was defined according to the ISTH criteria. 

Results:

Between 1990 and 2020, 35 patients met the inclusion criteria. Mean age was 58 years, and 51% was female. No underline cause was found in 82% of them. Major bleeding occurred in 31%, and haemostatic treatment was given to 82% patients. All subjects underwent IST, and monotherapy with oral steroids was the treatment of choice (71%). In 75% of the patients, pre-treatment IT was > 20 BU, and 57% had FVIII levels < 1%. Seventeen per cent did not achieve CR and, among those, 83% had initial IT > 20 BU and 66% had initial FVIII levels < 1%. No one died during observation time.

Conclusions:

AHA is a rare disease. In the present study, most of patients who did not achieve CR had high IT and very low FVIII levels, which suggests the prognostic role of these tests. 

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/acquired-haemophilia-a-data-from-a-regional-blood-center-in-rio-de-janeiro-brazil/