Abstract
Background and objectiveAcquired 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.MethodsWe 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.ResultsInitial 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.ConclusionsBased 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.
Highlights
Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder caused by the formation of neutralizing antibodies against endogenous factor VIII (FVIII)
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
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
Summary
Acquired hemophilia A (AHA) is an autoimmune disorder characterized by the formation of neutralizing autoantibodies against endogenous factor VIII (FVIII) [1]. The formation of these autoantibodies can be triggered by underlying risk factors such as malignancy, infection, concurrent autoimmune disease, lymphoproliferative disease, advanced age, pregnancy, as well as iatrogenic causes such as surgery, but is reported to be idiopathic in 51.9% of cases [1]. In the EACH2 cohort, AHA was typically found in older patients, with a reported median age of 73.9 years, a male predominance in approximately 57% of non-pregnancy-related cases, and a lack of personal or family history of bleeding [1]. Acquired hemophilia A (AHA) is an uncommon autoimmune bleeding disorder caused by the formation of neutralizing antibodies against endogenous factor VIII (FVIII). We aimed to analyze the impact of delays in diagnosis on AHA patients
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