Retrospective review of Acquired Haemophilia A from the largest Canadian Haemophilia treatment centre

Jayakar, Jai; O'Neill, Natalya E; Yan, Miu Chung; Nisenbaum, Rosane; Garvey, M. B.; Teitel, Jerome; Sholzberg, Michelle

doi:10.1111/hae.13598

Cited by 15 publications

(16 citation statements)

References 13 publications

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“…In line with many AHA registries, this study emphasizes the high rate of adverse events, especially infections. 5,15,17 This is however the first study evaluating risk-factors for treatment-related adverse events, which provides valuable information to guide therapy. In our analysis only age appeared to be significantly correlated with the overall adverse events rate.…”

Section: Discussionmentioning

confidence: 99%

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

et al. 2020

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Acquired hemophilia A (AHA) is a severe auto‐immune bleeding disorder. Treatment of AHA is burdensome and optimal management is still unresolved. Therefore a retrospective nationwide multi‐center cohort study (1992‐2018) was performed to evaluate clinical presentation and treatment efficacy and safety of AHA in the Netherlands. Multivariate logistic and Cox regression analysis was used to study independent associations between patient characteristics and clinical outcomes. A total of 143 patients (median age 73 years; 52.4% male) were included with a median follow‐up of 16.8 months (IQR 3.6‐41.5 months). First‐line immunosuppressive treatment was mostly steroid monotherapy (67.6%), steroids/cyclophosphamide (11.9%) and steroids/rituximab (11.9%), with success rates of 35.2%, 80.0% and 66.7% respectively, P < .05. Eventually 75% of patients achieved complete remission (CR). A high anti‐FVIII antibody titer, severe bleeding and steroid monotherapy were associated with lower CR rates. Infections, the most important adverse event, occurred significantly more often with steroid combination therapy compared to steroids alone (38.7% vs 10.6%; P = .001). Overall mortality was 38.2%, mostly due to infections (19.2%) compared to 7.7% fatal bleeds. Advanced age, underlying malignancy and ICU admission were predictors for mortality. This study showed that AHA is characterized by significant disease‐related and treatment‐related morbidity and mortality. A high anti‐FVIII titer, severe bleeding and steroid monotherapy were associated with a lower CR rate. The efficacy of steroid combination therapies however, was overshadowed by higher infection rates and infections represented the most important cause of death. The challenging and delicate balance between treatment effectivity and safety requires ongoing monitoring of AHA and further identification of prognostic markers.

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Section: Discussionmentioning

confidence: 99%

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

et al. 2020

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“…10 A recent single-centre, retrospective cohort study in Canada reported high rates of hospitalization (87.5%) and critical care admissions (12.5%) for patients with acquired hemophilia A. 11 Delays in time to treatment are associated with lower response rates and worse clinical outcomes. 4,8…”

Section: Discussionmentioning

confidence: 99%

New onset of acute heavy menstrual bleeding in a 34-year-old woman

Khalife¹,

Duffett²,

Wang³

et al. 2021

CMAJ

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“…The bleeding features of AHA are different from those of congenital hemophilia A. [ 14 ] In most cases of AHA, including the current patient, FVIII inhibitors lead to hemorrhage in the skin and mucous membranes, gastrointestinal bleeding, urinary tract bleeding, retroperitoneal bleeding, whereas hemarthrosis, a predominant characteristic of congenital FVIII defect, is not common. Congenital hemophilia A is an X chromosome-linked single-gene recessive hereditary bleeding disorder.…”

Section: Discussionmentioning

confidence: 99%

Acquired hemophilia A in a woman with systemic lupus erythematosus

Shen

et al. 2020

Medicine

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Rationale: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies directed against the activity of factor VIII (FVIII) and presents with prolonged bleeding. 5.7% of systemic lupus erythematosus (SLE) patients are affected by AHA. Patient concerns: A 51-year-old female patient with SLE presenting with the fatigue and spontaneous clinical bleeding symptoms such as hematuria and ecchymoses for 1 week. Diagnosis: Laboratory examinations revealed prolongation of the activated partial thromboplastin time (APTT) (65.7 s), decreased FVIII activity (1.4%), and a titer of FVIII inhibitors of 8.5 Bethesda units/mL. Interventions: Transfusion of recombinant human FVIII (ADVATE) in combination with intravenous methylprednisolone, cyclophosphamide, plasmapheresis, and fresh frozen plasma successfully stopped the bleeding and reduced the level of FVIII inhibitor. Outcomes: The size of the hematoma slowly decreased. The skin ecchymosis was gradually absorbed, the hemoglobin count increased, and the coagulation index gradually improved. There was no new bleeding or bleeding site. The patient was discharged and transferred to a local hospital for hospice care. Lessons: AHA in a patient with SLE is rare. Once it occurs, it can be life-threatening. Clinicians should remain aware that because some cases of AHA may have features of SLE, appropriate distinction and diagnosis of these different but associated diseases is necessary.

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Retrospective review of Acquired Haemophilia A from the largest Canadian Haemophilia treatment centre

Cited by 15 publications

References 13 publications

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

Treatment of acquired hemophilia A, a balancing act: results from a 27‐year Dutch cohort study

New onset of acute heavy menstrual bleeding in a 34-year-old woman

Acquired hemophilia A in a woman with systemic lupus erythematosus

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