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Acquired C1-inhibitor deficiency presenting with nephrotic syndrome

Lookup NU author(s): Dr Jamie Willows, Dr Katrina Wood, Dr Helen Bourne, Professor John SayerORCiD


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© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma. Both the acquired C1-INH deficiency and the nephrotic syndrome resolved when the underlying B-cell lymphoma was treated with rituximab and bendamustine, suggesting the underlying lymphoproliferative malignancy was driving both disorders.

Publication metadata

Author(s): Willows J, Wood K, Bourne H, Sayer JA

Publication type: Article

Publication status: Published

Journal: BMJ case reports

Year: 2019

Volume: 12

Issue: 7

Online publication date: 11/07/2019

Acceptance date: 26/06/2019

ISSN (electronic): 1757-790X

Publisher: NLM (Medline)


DOI: 10.1136/bcr-2019-230388

PubMed id: 31300605


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