Literature DB >> 28509101

Effectiveness of plasma exchange for acquired type A hemophilia.

Sawako Goto1, Susumu Ookawara2, Yuichiro Ueda1, Kiyonori Ito1, Haruhisa Miyazawa1, Taisuke Kitano1, Mitsutoshi Shindo1, Yoshio Kaku1, Keiji Hirai1, Taro Hoshino1, Masahiro Ashizawa3, Honami Mori1, Izumi Yoshida1, Kaoru Tabei1.   

Abstract

An 80-year-old man was treated at our hospital for chronic kidney disease, diabetes mellitus and hypertension. He presented a bleeding tendency over the past several weeks. When he was admitted to our hospital with a consciousness disturbance, he had irregular 10-20 cm patches of subcutaneous bleeding. Laboratory findings showed prolonged activated partial thromboplastin time (APTT) but normal platelet count and prothrombin time. The administration of fresh frozen plasma did not control the bleeding tendency and the result of APTT cross-mixing test suggested the presence of factor VIII inhibitor. The diagnosis of acquired hemophilia A was made by a marked decrease in factor VIII activity levels (<1 %) and a high titer of factor VIII inhibitor (11 Bethesda units). To remove the factor VIII inhibitor and suppress its production, plasma exchange was performed and methylprednisolone was administered. Thereafter, the bleeding tendency could be controlled with APTT measurement normalization. Plasma exchange does not have a first priority in the treatment but our case findings suggested that plasma exchange is an effective method for eliminating factor VIII inhibitor and alleviating acquired hemophilia A severity.

Entities:  

Keywords:  Acquired hemophilia A; Factor VIII inhibitor; Plasma exchange

Year:  2015        PMID: 28509101      PMCID: PMC5413767          DOI: 10.1007/s13730-015-0168-z

Source DB:  PubMed          Journal:  CEN Case Rep        ISSN: 2192-4449


  8 in total

Review 1.  Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors.

Authors:  Julio Delgado; Victor Jimenez-Yuste; Fernando Hernandez-Navarro; Ana Villar
Journal:  Br J Haematol       Date:  2003-04       Impact factor: 6.998

2.  Treatment of acquired hemophilia by the Bonn-Malmo Protocol: documentation of an in vivo immunomodulating concept.

Authors:  Heike Zeitler; Gudrun Ulrich-Merzenich; Lothar Hess; Eligius Konsek; Christoph Unkrig; Peter Walger; Hans Vetter; Hans-Hermann Brackmann
Journal:  Blood       Date:  2004-11-12       Impact factor: 22.113

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

Authors:  Peter W Collins; Sybil Hirsch; Trevor P Baglin; Gerard Dolan; John Hanley; Michael Makris; David M Keeling; Ri Liesner; Simon A Brown; Charles R M Hay
Journal:  Blood       Date:  2006-10-17       Impact factor: 22.113

Review 4.  The diagnosis and management of factor VIII and IX inhibitors: a guideline from the United Kingdom Haemophilia Centre Doctors Organisation.

Authors:  Charles R M Hay; S Brown; P W Collins; D M Keeling; R Liesner
Journal:  Br J Haematol       Date:  2006-06       Impact factor: 6.998

5.  Plasma exchange for acquired hemophilia: a case report.

Authors:  H Ogata; S Sakai; F Koiwa; H Tayama; E Kinugasa; T Ideura; T Akizawa
Journal:  Ther Apher       Date:  1999-11

Review 6.  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

7.  Immunoadsorption for pregnancy-associated severe acquired hemophilia.

Authors:  Heike Zeitler; Gudrun Ulrich-Merzenich; Natascha Marquardt; Johannes Oldenburg; Georg Goldmann
Journal:  Ther Apher Dial       Date:  2013-07-04       Impact factor: 1.762

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

Authors:  Angela Huth-Kühne; Francesco Baudo; Peter Collins; Jørgen Ingerslev; Craig M Kessler; Hervé Lévesque; Maria Eva Mingot Castellano; Midori Shima; Jean St-Louis
Journal:  Haematologica       Date:  2009-04       Impact factor: 9.941

  8 in total

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