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      A case of acquired hemophilia A in an elderly female

      case-report

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          ABSTRACT

          Acquired Hemophilia A (AHA) is a bleeding diathesis related to the development of factor VIII inhibitor, which can frequently go undetected. It commonly manifests as spontaneous mucosal bleeds without any known history of a bleeding disorder, but has the potential to cause life threatening bleeding especially in elderly patients with underlying comorbidities. Here we describe a case of AHA in a 78 year old female presenting with spontaneous mucocutaneous bleeding as tongue hematoma and recurrent gastrointestinal (GI) bleeding. Underlying etiology remained unclear in this case. While she did not require any reversal agents to control bleeding, the patient received steroids and rituximab as inhibitor eradication therapy.

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          Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation.

          Ri Liesner, Michael Makris, Sybil Hirsch … (2007)
          Acquired hemophilia A is a severe bleeding disorder caused by an autoantibody to factor VIII. Previous reports have focused on referral center patients and it is unclear whether these findings are generally applicable. To improve understanding of the disease, a 2-year observational study was established to identify and characterize the presenting features and outcome of all patients with acquired hemophilia A in the United Kingdom. This allowed a consecutive cohort of patients, unbiased by referral or reporting practice, to be studied. A total of 172 patients with a median age of 78 years were identified, an incidence of 1.48/million/y. The cohort was significantly older than previously reported series, but bleeding manifestations and underlying diseases were similar. Bleeding was the cause of death in 9% of the cohort and remained a risk until the inhibitor had been eradicated. There was no difference in inhibitor eradication or mortality between patients treated with steroids alone and a combination of steroids and cytotoxic agents. Relapse of the inhibitor was observed in 20% of the patients who had attained first complete remission. The data provide the most complete description of acquired hemophilia A available and are applicable to patients presenting to all centers.
          Article 0 comments Cited 145 times – based on 0 reviews     Review now
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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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                Author and article information

                Journal
                Journal ID (nlm-ta): J Community Hosp Intern Med Perspect
                Journal ID (iso-abbrev): J Community Hosp Intern Med Perspect
                Journal ID (publisher-id): ZJCH
                Journal ID (publisher-id): zjch20
                Title: Journal of Community Hospital Internal Medicine Perspectives
                Publisher: Taylor & Francis
                ISSN (Electronic): 2000-9666
                Publication date Collection: 2018
                Publication date (Electronic): 23 August 2018
                Volume: 8
                Issue: 4
                Pages: 237-240
                Affiliations
                [a ]Department of Internal Medicine, Saint Agnes Hospital , Baltimore, MD, USA
                [b ]Department of Hematology and Oncology, Saint Agnes Hospital , Baltimore, MD, USA
                Author notes
                CONTACT Kiranveer Kaur drkiranveer@ 123456gmail.com Department of Hematology and Oncology, Saint Agnes Hospital , 900 S Caton Avenue, Baltimore, MD21229, USA
                Article
                Publisher ID: 1487246
                DOI: 10.1080/20009666.2018.1487246
                PMC ID: 6116292
                PubMed ID: 30181835
                SO-VID: 6a432233-50ac-4b3d-b26c-3dc5a145b507
                Copyright © © 2018 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group on behalf of Greater Baltimore Medical Center.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License ( http://creativecommons.org/licenses/by-nc/4.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 18 April 2018
                Date accepted : 30 May 2018
                Page count
                Figures: 1, References: 21, Pages: 4
                Categories
                Subject: Case Report

                Keywords: acquired hemophilia a,bleeding,factor viii inhibitor,rituximab
                Data availability:
                Keywords: acquired hemophilia a, bleeding, factor viii inhibitor, rituximab

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