Acquired Factor VIII Inhibitor as Initial Manifestation of Collagen Vascular Disease: Response to Combination Immunosuppression as First-Line Therapy

Wilson, Bethany; Means, Robert T.

doi:10.1097/maj.0b013e31820f28ab

Cited by 4 publications

(2 citation statements)

References 18 publications

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“…As 20% of patients experience a relapse within a year following therapy, close monitoring is paramount. There is some evidence that the use of rituximab as part of a combined immunosuppression regimen may be more effective as a first‐line approach to AHA.…”

Section: Discussionmentioning

confidence: 99%

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Acquired haemophilia A: a clinical case

2012

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Background Acquired haemophilia A (AHA) is a rare medical emergency that may be characterised by severe and life‐threatening haemorrhage. Aims This case report highlights the need for a better awareness of this disorder in terms of diagnosis and urgent management. Materials and Methods This is a clinical presentation of a 77‐year‐old Caucasian man who presented to Accident and Emergency with profuse bleeding from his tongue, secondary to mechanical trauma. Local haemostatic measures were only temporarily effective as he returned on a further five occasions over a 3‐week period with recurrent bleeding from the same site. A coagulation screen revealed prolongation of the activated partial thromboplastin time that did not correct on a 50/50 mixing study with normal plasma. Subsequent analysis of intrinsic coagulation factor activity levels revealed severe factor VIII (FVIII) deficiency. A high‐titre FVIII inhibitor was identified in his plasma and the diagnosis of AHA was made. The patient was promptly transferred to the regional haemophilia comprehensive care centre for specialist management. Discussion AHA should be suspected in a patient with no personal or family history of a bleeding disorder who presents with soft tissue haemorrhage unresponsive to local measures. Conclusion This condition is particularly relevant to dental clinicians as patients may present with oral clinical signs of disease. In addition, affected individuals are also at risk of severe haemorrhage following accidental trauma or invasive oral surgery procedures.

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Section: Discussionmentioning

confidence: 99%

“…Although many achieve a second complete remission, long-term immunosuppression may be indicated in cases 22 . Our patient remains in remission, 11 months following chemotherapy, on long-term maintenance steroids with a FVIII level of 181%.…”

Section: Discussionmentioning

confidence: 99%

Acquired haemophilia A: a clinical case

2012

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Rituximab for eradicating inhibitors in people with acquired haemophilia A

Zeng

Zhou

Duan

et al. 2016

Cochrane Database of Systematic Reviews

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No randomised clinical trials of rituximab for acquired hemophilia A were found. Thus, based on the highest quality of evidence, we are not able to draw any conclusions or make any recommendations on rituximab for eradicating inhibitors in people with acquired haemophilia A. Given that undertaking randomised controlled trials in this field is a complex task, the authors suggest that, while planning such trials, clinicians treating the disease continue to base their choices on alternative, lower quality sources of evidence. The authors plan, for a future update of this review, to appraise and incorporate any randomised controlled trials, as well as other high-quality non-randomised studies.

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Rituximab for eradicating inhibitors in people with acquired haemophilia A

Remmington

Smith

2021

Cochrane Database of Systematic Reviews

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Acquired Factor VIII Inhibitor as Initial Manifestation of Collagen Vascular Disease: Response to Combination Immunosuppression as First-Line Therapy

Cited by 4 publications

References 18 publications

Acquired haemophilia A: a clinical case

Acquired haemophilia A: a clinical case

Rituximab for eradicating inhibitors in people with acquired haemophilia A

Rituximab for eradicating inhibitors in people with acquired haemophilia A

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