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  1. bookVolume 29 (2021): Issue 4 (October 2021)
Revista Romana de Medicina de Laborator's Cover Image
Revista Romana de Medicina de Laborator
Journal Details
License
Format
Journal
First Published
08 Aug 2013
Publication timeframe
4 times per year
Languages
English
access type Open Access

Acquired Angioedema Due to C1 inhibitor Deficiency Caused by Non-Hodgkin Lymphoma in a Patient with Myasthenia Gravis

Noémi Bara,Noémi Bara
Valentin Nadasan,Valentin Nadasan
Lilian Varga andLilian Varga
Henriette FarkasHenriette Farkas
Published Online: 22 Oct 2021
Page range: 453 - 456
Received: 07 Sep 2021
Accepted: 23 Sep 2021
Journal Details
License
Format
Journal
First Published
08 Aug 2013
Publication timeframe
4 times per year
Languages
English
Abstract

Acquired angioedema due to C1-inhibitor deficiency is a very rare disorder that usually appears in patients with lymphoproliferative and/or autoimmune diseases. This type of swelling is bradykinin mediated and does not respond to antihistamines, corticosteroids, or epinephrine. The symptoms usually appear in patients older than 40 years with recurrent episodes of angioedema without wheals. The family history is negative. The swelling could affect any tissue, but most frequently is located at the face, lips, tongue, larynx, or extremities. In the gastrointestinal tract, it causes pain, nausea, vomiting, and diarrhea. The upper respiratory airway oedema is a potentially life-threatening condition due to asphyxiation. The oedema attacks may precede the symptoms of the causative disease for months or years. In most cases, the treatment of the underlying disease resolves the angioedema episodes. Here we report a case of C1-INH-AAE caused by non-Hodgkin lymphoma in a patient diagnosed many years before with myasthenia gravis whose angioedema symptoms resolved after the specific treatment of lymphoma.

Keywords

  • rare disease
  • bradykinin mediated angioedema
  • lymphoproliferative disease
  • autoimmune disease
  • angioedema attack

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