Literature DB >> 32063777

Acquired hemophilia A.

Yadav Pandey1, Dinesh Atwal2, Manojna Konda1, Arya Roy1, Appalanaidu Sasapu2.   

Abstract

Acquired inhibitors of coagulation are a group of rare but potentially life-threatening blood disorders characterized by the presence of autoantibodies directed against clotting factor. Autoantibody against factor VIII is the most common form of clotting factor inhibitor, a condition also known as acquired hemophilia A. We present a clinical series of nine patients diagnosed and treated for acquired hemophilia A at our institution. Among these nine patients, there were five men and four women with a median age of 64 years. All patients presented with bleeding diathesis. Factor eight inhibitor bypassing agent and/or recombinant factor VIIa were predominantly used for control of active bleeding. For elimination of autoantibodies, either steroids alone or the combination of steroids with rituximab or oral cyclophosphamide was used. Despite aggressive measures, two of the patients had a poor outcome; seven of the nine patients (77%) had a good clinical outcome. Acquired hemophilia A should be strongly suspected in any patient presenting with bleeding and a prolonged activated partial thromboplastin time. Early initiation of factor bypassing agents such as activated prothrombin complex concentrates or recombinant factor VIIa, along with the use of immunosuppressive agents, can be lifesaving.
Copyright © 2020 Baylor University Medical Center.

Entities:  

Keywords:  Acquired hemophilia A; acquired inhibitors of coagulation; activated prothrombin complex concentrates; recombinant factor VIIa

Year:  2019        PMID: 32063777      PMCID: PMC6988643          DOI: 10.1080/08998280.2019.1689024

Source DB:  PubMed          Journal:  Proc (Bayl Univ Med Cent)        ISSN: 0899-8280


  21 in total

1.  New perspectives in hemophilia treatment.

Authors:  Craig M Kessler
Journal:  Hematology Am Soc Hematol Educ Program       Date:  2005

2.  The efficacy of rituximab in the treatment of inhibitor-associated hemostatic disorders.

Authors:  Massimo Franchini; Dino Veneri; Giuseppe Lippi; Rachel Stenner
Journal:  Thromb Haemost       Date:  2006-08       Impact factor: 5.249

3.  Anti-idiotypic suppression of autoantibodies to factor VIII (antihaemophilic factor) by high-dose intravenous gammaglobulin.

Authors:  Y Sultan; M D Kazatchkine; P Maisonneuve; U E Nydegger
Journal:  Lancet       Date:  1984-10-06       Impact factor: 79.321

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

Authors:  Francesco Baudo; Peter Collins; Angela Huth-Kühne; Hervé Lévesque; Pascual Marco; László Nemes; Fabio Pellegrini; Lilian Tengborn; Paul Knoebl
Journal:  Blood       Date:  2012-05-22       Impact factor: 22.113

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

Authors:  P Knoebl; P Marco; F Baudo; P Collins; A Huth-Kühne; L Nemes; F Pellegrini; L Tengborn; H Lévesque
Journal:  J Thromb Haemost       Date:  2012-04       Impact factor: 5.824

Review 6.  Treatment of acquired haemophilia with recombinant activated FVII: a critical appraisal.

Authors:  M J Sumner; B D Geldziler; M Pedersen; S Seremetis
Journal:  Haemophilia       Date:  2007-09       Impact factor: 4.287

7.  Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia.

Authors:  Roberto Stasi; Maurizio Brunetti; Elisa Stipa; Sergio Amadori
Journal:  Blood       Date:  2004-03-02       Impact factor: 22.113

Review 8.  Advances in the understanding of acquired haemophilia A: implications for clinical practice.

Authors:  Peter W Collins; Charles L Percy
Journal:  Br J Haematol       Date:  2009-10-07       Impact factor: 6.998

9.  FEIBA in the treatment of acquired haemophilia A: results from the prospective multicentre French 'FEIBA dans l'hémophilie A acquise' (FEIBHAC) registry.

Authors:  J-Y Borg; C Négrier; I Durieu; E Dolimier; A-M Masquelier; H Lévesque
Journal:  Haemophilia       Date:  2014-10-31       Impact factor: 4.287

10.  A Case of a Patient Who Is Diagnosed with Mild Acquired Hemophilia A after Tooth Extraction Died of Acute Subdural Hematoma due to Head Injury.

Authors:  Tomohisa Kitamura; Tsuyoshi Sato; Eiji Ikami; Yosuke Fukushima; Tetsuya Yoda
Journal:  Case Rep Dent       Date:  2018-12-09
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