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Identifying risk factors and optimizing standard of care for patients with acquired haemophilia A: Results from a Czech patient cohort.
- Source :
-
Haemophilia : the official journal of the World Federation of Hemophilia [Haemophilia] 2020 Jul; Vol. 26 (4), pp. 643-651. Date of Electronic Publication: 2020 Jun 26. - Publication Year :
- 2020
-
Abstract
- Introduction: Acquired haemophilia A (AHA) is a rare autoimmune disorder, characterized by bleeds of varying severity caused by autoantibodies against factor VIII (FVIII).<br />Aim: Identify risk factors associated with AHA-related deaths/relapses and assess the effect of increased corticosteroid doses.<br />Methods: AHA patients treated across two specialist centres in the Czech Republic, generally receiving first-line haemostatic therapy with rFVIIa and immunosuppression with corticosteroids/cyclophosphamide, were included. We analysed the association between early death (within 8 weeks of diagnosis [considered disease-related]) and age, malignancy, FVIII levels and bleeding severity. Risk factors associated with reduced 2-year survival and relapse incidence, and the effect of increased corticosteroid doses on early death and remission were also assessed.<br />Results: The demographics of the described cohort (n = 66) were similar to other AHA registries. Early death occurred in 20% of cases. Unlike age and malignancy, FVIII levels <1% and severe bleeding were associated significantly with early death (P = .010 and P = .046, respectively). Patients with underlying malignancy or requiring continued haemostatic therapy exhibited significantly decreased 2-year survival compared with those without these risk factors (P = .007 and P = .006, respectively). Patients with an underlying autoimmune disease relapsed significantly more than those without (P = .015). Higher corticosteroid doses were associated with a significantly increased incidence of early deaths (P < .001), but also with early remission (P < .001).<br />Conclusion: Based on this rather large patient cohort, we were able to evaluate the significance of several risk factors associated with treatment outcomes in AHA and the effect of initial treatment with corticosteroids on survival and time to remission.<br /> (© 2020 John Wiley & Sons Ltd.)
- Subjects :
- Adrenal Cortex Hormones adverse effects
Adrenal Cortex Hormones therapeutic use
Adult
Aged
Aged, 80 and over
Autoantibodies immunology
Autoimmune Diseases complications
Cohort Studies
Czech Republic epidemiology
Factor VIII immunology
Factor VIII metabolism
Factor VIIa administration & dosage
Factor VIIa therapeutic use
Female
Hemophilia A complications
Hemophilia A mortality
Hemorrhage etiology
Hemorrhage immunology
Hemorrhage mortality
Humans
Immunosuppressive Agents administration & dosage
Immunosuppressive Agents therapeutic use
Male
Middle Aged
Recombinant Proteins administration & dosage
Recombinant Proteins therapeutic use
Recurrence
Remission Induction
Risk Factors
Severity of Illness Index
Standard of Care trends
Survival Analysis
Treatment Outcome
Factor VIII antagonists & inhibitors
Hemophilia A drug therapy
Hemorrhage prevention & control
Standard of Care statistics & numerical data
Subjects
Details
- Language :
- English
- ISSN :
- 1365-2516
- Volume :
- 26
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Haemophilia : the official journal of the World Federation of Hemophilia
- Publication Type :
- Academic Journal
- Accession number :
- 32590889
- Full Text :
- https://doi.org/10.1111/hae.14084