Acquired haemophilia in cancer: A systematic and critical literature review

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Abstract

There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer.

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Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2).

P Knoebl, Alan Marco, F Baudo … (2012)

Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA.

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Management of bleeding in acquired hemophilia A: results from the European Acquired Haemophilia (EACH2) Registry.

Francesco Baudo, Peter Collins, Angela Huth-Kühne … (2012)

Acquired hemophilia A is a rare bleeding disorder caused by autoantibodies to coagulation FVIII. Bleeding episodes at presentation are spontaneous and severe in most cases. Optimal hemostatic therapy is controversial, and available data are from observational and retrospective studies only. The EACH2 registry, a multicenter, pan-European, Web-based database, reports current patient management. The aim was to assess the control of first bleeding episodes treated with a bypassing agent (rFVIIa or aPCC), FVIII, or DDAVP among 501 registered patients. Of 482 patients with one or more bleeding episodes, 144 (30%) received no treatment for bleeding; 31 were treated with symptomatic therapy only. Among 307 patients treated with a first-line hemostatic agent, 174 (56.7%) received rFVIIa, 63 (20.5%) aPCC, 56 (18.2%) FVIII, and 14 (4.6%) DDAVP. Bleeding was controlled in 269 of 338 (79.6%) patients treated with a first-line hemostatic agent or ancillary therapy alone. Propensity score matching was applied to allow unbiased comparison between treatment groups. Bleeding control was significantly higher in patients treated with bypassing agents versus FVIII/DDAVP (93.3% vs 68.3%; P = .003). Bleeding control was similar between rFVIIa and aPCC (93.0%; P = 1). Thrombotic events were reported in 3.6% of treated patients with a similar incidence between rFVIIa (2.9%) and aPCC (4.8%).

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International recommendations on the diagnosis and treatment of patients with acquired hemophilia A.

Angela Huth-Kühne, Francesco Baudo, Peter Collins … (2009)

Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies directed against circulating coagulation factor (F) VIII. Typically, patients with no prior history of a bleeding disorder present with spontaneous bleeding and an isolated prolonged aPTT. AHA may, however, present without any bleeding symptoms, therefore an isolated prolonged aPTT should always be investigated further irrespective of the clinical findings. Control of acute bleeding is the first priority, and we recommend first-line therapy with bypassing agents such as recombinant activated FVII or activated prothrombin complex concentrate. Once the diagnosis has been achieved, immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. We recommend initial treatment with corticosteroids or combination therapy with corticosteroids and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy fails or is contraindicated. In contrast to congenital hemophilia, no comparative studies exist to support treatment recommendations for patients with AHA, therefore treatment guidance must rely on the expertise and clinical experience of specialists in the field. The aim of this document is to provide a set of international practice guidelines based on our collective clinical experience in treating patients with AHA and contribute to improved care for this patient group.

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Author and article information

Journal

Title: Haemophilia

Abbreviated Title: Haemophilia

Publisher: Wiley

ISSN: 13518216

Publication date Created: January 2018

Publication date (Print): January 2018

Publication date (Electronic): September 27 2017

Volume: 24

Issue: 1

Pages: 43-56

Affiliations

[1 ]Hematology Unit, Thrombosis and Hemostasis Reference Regional Center; Università degli studi di Palermo; Palermo Italy

[2 ]Lombardi Comprehensive Cancer Center; Georgetown University Medical Center; Washington DC USA

Article

DOI: 10.1111/hae.13355

PubMed ID: 28960809

SO-VID: 50bc1612-5953-4209-bb84-216b69d5dd43

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http://doi.wiley.com/10.1002/tdm_license_1.1

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