Literature DB >> 30882350

[Acquired hemophilia A associated with bullous pemphigoid and multiple myeloma: a case report].

Élise Sourdeau1, Sylvain Clauser2, Romain Prud'Homme3, Valérie Bardet2, Leyla Calmette1.   

Abstract

Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disorder caused by circulating autoantibodies directed against factor (F) VIII. Apart from idiopathic cases, AHA is associated with autoimmune diseases, cancers, use of medications, pregnancy and the post-partum period. We report the case of a 78-year-old a male patient presenting with symptoms of a hematoma after a fall three days previously. He is medically followed for multiple myeloma and bullous pemphigoid. Laboratory investigations revealed isolated and recent increased of activated partial thromboplastin time (80,6/32,1s) and a markedly low FVIII activity (< 1%). The high-titer of FVIII inhibitor (19 Bethesda units/mL) confirmed the diagnosis of AHA diagnosis. The symptoms were noticeably alleviated following bortezomib, cyclophosphamide and dexamethasone therapy. This report describes a rare case of AHA associated with bullous pemphigoid and multiple myeloma. These pathologies induce an immunity modification that can predispose or be associated with the development of anti-FVIII inhibitors. This case illustrates two possible physiopathological hypotheses for the development of AHA, which will be discussed in this case report.

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Keywords:  acquired hemophilia A; bullous pemphigoid; myeloma

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Year:  2019        PMID: 30882350     DOI: 10.1684/abc.2018.1405

Source DB:  PubMed          Journal:  Ann Biol Clin (Paris)        ISSN: 0003-3898            Impact factor:   0.459


  1 in total

Review 1.  Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature.

Authors:  Katarzyna A Jalowiec; Martin Andres; Behrouz Mansouri Taleghani; Albulena Musa; Martina Dickenmann; Anne Angelillo-Scherrer; Alicia Rovó; Johanna A Kremer Hovinga
Journal:  J Med Case Rep       Date:  2020-10-30
  1 in total

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