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Management of acquired haemophilia A in a Portuguese Center: 16 years and 16 cases

1Congenital Coagulopathies Reference Centre- Centro Hospitalar Universitário Lisboa Norte - Hospital Santa Maria, Lisbon, Portugal, Lisbon, Lisboa, Portugal, 2Congenital Coagulopathies Reference Centre- Centro Hospitalar Universitário Lisboa Norte - Hospital Santa Maria, Lisbon, Portugal, Lisboa, Lisboa, Portugal, 3Hospital do Espirito Santo - Évora, Évora, Evora, Portugal, 4Centro Hospitalar Universitário Lisboa Norte - Hospital Santa Maria, Lisbon, Portugal, Lisboa, Lisboa, Portugal, 5Centro Hospitalar Lisboa Ocidental, Hospital de São Francisco Xavier, Lisboa, Lisboa, Lisboa, Portugal, 6Hospital Nélio Mendonça, Funchal, Madeira, Portugal, 7Centro Hospitalar Universitário Lisboa Norte - Hospital Santa Maria, Lisboa, Lisboa, Portugal

Abstract Number: PB0001

Meeting: ISTH 2022 Congress

Theme: Acquired Bleeding Disorders » Management/Treatments of Acquired Bleeding

Background: Management of acquired haemophilia A (AHA), a rare and severe autoimmune disease, is based in the control of bleeding, eradication of inhibitors and diagnosis of the underlying cause.

Aims: We, retrospectively, analyzed demographics, time for diagnosis and for eradication of inhibitors, symptoms, haemostatic and immunosuppressive therapy, prognosis, and underlying cause.

Methods: Between 2006 and 2021, 16 patients (9 males; 7 females), median age 76 years (62-90) were diagnosed at our Center.

Results: Time from onset of symptoms to diagnosis was less than 1 week in 7 patients, between 1 and 4 weeks in 5 and more than 4 weeks in 3.

Median FVIII was 3,5% ( < 1-21) and median higher inhibitor titer was 193 BU (3 -1250). Symptoms observed were muscular (11); subcutaneous (3); oropharyngeal (2); urinary (3); intracranial (1) and gastrointestinal (1). In 3 patients bleeding was related to surgical procedures. Hemostatic therapy was needed in 13 patients. Recombinant activated factor VII (rFVIIa) alone was effective in 9 /12 patients. In 1 patient, with a life-threatening hemorrhage, association with activated prothrombin complexes (aPCC) was needed. aPCC alone was effective in another patient. No adverse events related to haemostatic treatment were observed. Eight patients (50%) received other blood components. In 10/11 patients who fully recovered, immunosuppression was done with corticoids only. In one patient, due to the severity of bleeding, cyclophosphamide and rituximab were associated with good results. Median time to remission was 26 days and no relapse was observed. AHA was classified as idiopathic in 11/16 (62,5%) patients. In the other cases, AHA was related to Epstein- Barr virus (1); malignancy (2) and autoimmune disease (2). Mortality was observed in 5/ 16 patients, being bleeding the cause of death in 3 patients.

Conclusion(s): Diagnosis, treatment and prognosis of AHA is influenced by the timing of diagnosis and adequate and effective treatment.

Table

Clinical cases of acquired haemophilia A

To cite this abstract in AMA style:

Catarino C, Rodrigues F, Pereira A, Pestana J, Peixoto C, Campaniço S, Parusnikova L, Correia C, Pereira C, Parcelas E, Lucas J, Passos D, Croca S, Afonso P, Morgado P, Calisto C, Cruz S, Carriço M. Management of acquired haemophilia A in a Portuguese Center: 16 years and 16 cases [abstract]. https://abstracts.isth.org/abstract/management-of-acquired-haemophilia-a-in-a-portuguese-center-16-years-and-16-cases/. Accessed October 1, 2023.

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