Clinical evaluation of bleeds and response to haemostatic treatment in patients with acquired haemophilia: A global expert consensus statement

Background Acquired haemophilia (AH) is a rare bleeding disorder with significant morbidity and mortality. Most patients initially present to physicians without experience of the disease, delaying diagnosis and potentially worsening outcomes. Existing guidance in AH is limited to clinical opinion of...

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Bibliographic Details
Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2019-11, Vol.25 (6), p.969-978
Main Authors: Tiede, Andreas, Giangrande, Paul, Teitel, Jerome, Amano, Kagehiro, Benson, Gary, Nemes, László, Jiménez‐Yuste, Victor, d'Oiron, Roseline, Benchikh El Fegoun, Soraya, Kessler, Craig M.
Format: Article
Language:English
Subjects:
Acquired haemophilia
Bleeding
coagulation
Consensus
Delphi methodology
Delphi Technique
diagnosis and management
expert consensus
Expert Testimony
Female
Gastrointestinal tract
Hematology
Hemophilia
Hemophilia A - complications
Hemorrhage - complications
Hemorrhage - drug therapy
Hemostatics - pharmacology
Hemostatics - therapeutic use
Human health and pathology
Humans
Internationality
Life Sciences
Male
Morbidity
Muscles
Patients
Pharynx
Postpartum
rare bleeding disorders
Surveys and Questionnaires
Treatment Outcome
Urinary tract
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Summary:Background Acquired haemophilia (AH) is a rare bleeding disorder with significant morbidity and mortality. Most patients initially present to physicians without experience of the disease, delaying diagnosis and potentially worsening outcomes. Existing guidance in AH is limited to clinical opinion of few experts and does not address monitoring bleeds in specific anatomical locations. Aim Derive consensus from a large sample of experts around the world in monitoring bleeding patients with AH. Methods Using the Delphi methodology, a structured survey, designed to derive consensus on how to monitor bleeding patients with AH, was developed by a steering committee for completion by a group of haematologists with an interest in AH. Consensus was defined as ≥75% agreement with a given survey statement. After three rounds of survey refinement, a final list of consensus statements was compiled. Results Thirty‐six global specialists in AH participated. The participants spanned 20 countries and had treated a median of 12.0 (range, 1‐50) patients with AH within the preceding 5 years. Consensus was achieved in all items after three survey rounds. In addition to statements on general management of bleeding patients, consensus statements in the following areas were presented: urinary tract, gastrointestinal tract, muscles, skin, joints, nose, pharynx, mouth, intracranial and postpartum. Conclusions Here, we present consensus statements derived from a broad sample of global specialists to address monitoring of location‐specific bleeds and evaluating efficacy of bleeding treatment in patients with AH. These statements could be applied in practice by treating physicians and validated by individual population surveys.
ISSN:1351-8216
1365-2516
DOI:10.1111/hae.13844