Time is Blood: The Impact of Diagnostic Delays on Acquired Hemophilia A.
acquired hemophilia a
aha
bleeding
diagnostic delays
factor viii inhibitor
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
accepted:
09
02
2022
entrez:
28
3
2022
pubmed:
29
3
2022
medline:
29
3
2022
Statut:
epublish
Résumé
Background and objective Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder caused by the formation of neutralizing antibodies against endogenous factor VIII (FVIII). Delays between the onset of symptoms and the correct diagnosis of the condition lead to poor outcomes and a higher mortality rate. In this study, we aimed to analyze the impact of delays in diagnosis on AHA patients. Methods We conducted a retrospective study at a single hospital system between March 1, 2010, and January 17, 2017, which included six patients meeting the criteria for AHA diagnosis. Results Initial analysis revealed a median age of 79.5 years and a median time to diagnosis from the onset of bleeding of 14 days. Among the six patients, three had cancer (bladder, renal, and prostate) and three had unknown etiologies. One of the patients died prior to the initiation of a bypassing agent. The remaining five patients received recombinant FVIIa (NovoSeven®, Novo Nordisk, Bagsværd, Denmark), and two of those five required a second-line bypassing agent, recombinant porcine sequence FVIII (Obizur®, Takeda Pharmaceutical, Tokyo, Japan) for refractory bleeding. All five patients achieved hemostasis; however, three died within a year, and none of the patients survived for five years. Four of these five patients died directly from bleeding complications. Conclusions Based on our study findings and review of the literature, we propose an algorithm to potentially aid in the early diagnosis and treatment of AHA in emergency and non-specialized settings.
Identifiants
pubmed: 35340501
doi: 10.7759/cureus.22048
pmc: PMC8915674
doi:
Types de publication
Journal Article
Langues
eng
Pagination
e22048Informations de copyright
Copyright © 2022, Fragner et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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