Barriers to effective diagnosis and management of a bleeding patient with undiagnosed bleeding disorder across multiple specialties: results of a quantitative case-based survey

Reding, Mark T.; Cooper, David L.

doi:10.2147/jmdh.s35272

Cited by 19 publications

(28 citation statements)

References 27 publications

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“…Of those, emergency medicine physicians were least likely to consult hematology. 1 The reasons why bleeding disorders in the aging are overlooked could be due to the fact that aging is usually associated with thrombotic rather than hemorrhagic complications. Early signs of a bleeding disorder such as bruising are easily dismissed as "senile purpura" or "normal" side effects of drugs they are taking such as antiplatelet agents and anticoagulants.…”

Section: Learning Objectivesmentioning

confidence: 99%

Acquired bleeding disorders in the elderly

Kruse‐Jarres

2015

Hematology

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The hemostatic balance changes with advancing age which may be due to factors such as platelet activation, increase of certain clotting factor proteins, slowing of the fibrinolytic system, and modification of the endothelium and blood flow. Generally, this predisposes the elderly to thrombosis rather than bleeding. It often necessitates antiplatelet or anticoagulation therapy, which can cause significant bleeding problems in an aging population. Additionally, changing renal function, modification in immune regulation, and a multitude of other disease processes, can give rise to acquired bleeding disorders. Bleeding can prove difficult to treat in a dynamic environment and in a population that may have underlying thrombotic risk factors.This article discusses some specific challenges of acquired bleeding arising in the elderly. The use of anticoagulation and nonsteroidal anti-inflammatory medications is prevalent in the treatment of the elderly and predisposes them to increased bleeding risk as their physiology changes. When prescribing and monitoring these therapies, it is exceedingly important to weigh thrombotic versus bleeding risks. There are additional rare acquired bleeding disorders that predominantly affect the elderly. One of them is acquired hemophilia, which is an autoimmune disorder arising from antibodies against factor VIII. The treatment challenge rests in the use of hemostatic agents in a population that is already at increased risk for thrombotic complications. Another rare disorder of intensifying interest, acquired von Willebrand syndrome, has a multitude of etiologic mechanisms. Understanding the underlying pathophysiology is essential in making a treatment decision for this disorder. Learning Objectives• To recognize that coagulation changes with age and that it may predispose elderly patients on antithrombotic therapies to bleeding • To recognize the underlying thrombotic risk and comorbidities complicating the treatment of acquired hemophilia A • To understand the various pathophysiologic mechanisms leading to Acquired Von Willebrand Syndrome and assign appropriate treatment according to that mechanism Acquired bleeding disorders in the aging population are of concern because they are not readily diagnosed. In a study, community physicians from various specialties were presented with a hypothetical older adult woman complaining of recurrent epistaxis. Although most practitioners ordered complete blood counts and activated partial thromboplastin time (aPTT) as part of their work-up, less than one-half of nonhematologists repeated a significantly prolonged aPTT and Ͻ45% consulted a hematologist. Of those, emergency medicine physicians were least likely to consult hematology. 1

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Section: Learning Objectivesmentioning

confidence: 99%

Acquired bleeding disorders in the elderly

Kruse‐Jarres

2015

Hematology

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“…The diagnosis and management of AHA is complex and ideally should be coordinated by haemophilia centres with clinicians and laboratory staff experienced in the management of inhibitors . A recent quantitative case‐based survey of 302 physicians across a range of specialties (haematology, emergency medicine, geriatrics, internal medicine, rheumatology and critical care medicine) illustrated a general lack of consideration and response to the presenting symptom of bleeding and prolonged aPTT in AHA . To provide an educational resource to support the evaluation of laboratory results as part of the diagnosis of bleeding disorders, an iOS/Android/Web/Desktop application (app) has been developed.…”

Section: Diagnosis Of Ahamentioning

confidence: 99%

Acquired haemophilia: an overview for clinical practice

Kessler

Knöbl

2015

European J of Haematology

115

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Acquired haemophilia is a potentially life-threatening bleeding disorder caused by the development of autoantibodies against coagulation factors, most commonly against factor (F) VIII (acquired haemophilia A; AHA). In around half of patients, an underlying disorder is associated with AHA; the remaining cases are idiopathic. Typically, the disorder presents with bleeding, ranging from mild to life- and limb-threatening, in patients with no personal or family bleeding history. Diagnosis involves an isolated prolongation of the activated partial thromboplastin time, without correction in mixing studies, low FVIII activity levels and evidence of a FVIII inhibitor. As AHA is rare, a lack of familiarity of the condition may result in delayed diagnosis, and prompt haemostatic control is required to reduce morbidity and mortality. Bypassing agents (recombinant activated factor VII or activated prothrombin complex concentrates) can be used to control acute bleeding, and immunosuppression is necessary to eradicate the inhibitor. As clinical trials in this rare and heterogeneous disease are difficult, current evidence comes from observational studies, including registries. This review will focus on the diagnostic and therapeutic challenges of AHA and summarise how understanding of this complex condition has increased based on recent registry data.

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“…1 Due to the potential high morbidity and mortality, it is important that diagnosis and management of AH is undertaken quickly. 9 There are only a small number of specialists experienced in diagnosis and treatment of this condition, and they are based almost exclusively in academic tertiary centres. 4 While it did not affect patient outcome in this study, it was acknowledged that patients were still at increased risk of fatal bleeds until eradication of the autoantibody.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2019

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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.

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Barriers to effective diagnosis and management of a bleeding patient with undiagnosed bleeding disorder across multiple specialties: results of a quantitative case-based survey

Cited by 19 publications

References 27 publications

Acquired bleeding disorders in the elderly

Acquired bleeding disorders in the elderly

Acquired haemophilia: an overview for clinical practice

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

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